Aim and background: Adrenal lipomas are very rare benign tumors of adrenal gland. Most of these are non-functional and asymptomatic, with most of them being diagnosed on autopsy or as an incidentaloma on imaging. There are no cases being reported of adrenal lipoma associated with hormonal secretion in the literature until now. Primary hyperaldosteronism is one of the common causes of resistant hypertension which can increase the cardiovascular morbidity and mortality. It is usually caused by an adrenal adenoma, unilateral or bilateral adrenal hyperplasia (BAH), or in rare cases adrenal carcinoma or inherited conditions of familial hyperaldosteronism. It is important to diagnose it appropriately as it can be treated effectively with either medical or surgical modalities. We report a rare case, probably the first case, of association of adrenal lipoma with primary hyperaldosteronism, who was treated successfully with surgery. Case description: A 57 years old female, with uncontrolled type 2 diabetes mellitus and hypertension, was admitted with right lower limb cellulitis and lower respiratory tract infection. She was on three antihypertensive medications and yet the blood pressure was high (180/100 mm Hg). Her initial blood investigations during admission showed: HbA1c–9.5%, serum potassium–4.1 mmoL/L, serum creatinine–0.8 mg/dL. Computed tomography of the chest, which was done for her lung infection, revealed a lipid rich right adrenal adenoma (incidentaloma), of size 21 × 17 mm. Clinically, she did not have any specific symptoms or signs of Cushing syndrome, pheochromocytoma or primary hyperaldosteronism, except for resistant hypertension. There was no clinical evidence of adrenal insufficiency either. Baseline 8 am cortisol was found to be normal-22.93 μg/dL. The 2D echocardiogram was normal except for grade I diastolic dysfunction. She was treated for her infection, following which she recovered and discharged. Hormonal evaluation for right adrenal adenoma was done later during follow-up. The investigations showed: 8 am cortisol–14.9 μg/dL, potassium–4.1 mmoL/L; post over-night dexamethasone suppression test cortisol–1.7 μg/dL, plasma free metanephrines–89.3 pg/mL (<100), plasma free normetanephrines–472 pg/mL (216), 24 hours urinary normetanephrines–91.26 μg, plasma renin activity (PRA)–0.32 ng/mL/hour, and serum aldosterone–12.1 ng/dL. The aldosterone renin ratio (ARR) was 37.81, which was found to be elevated and suggestive of primary hyperaldosteronism. Then she underwent confirmatory test in the form of saline infusion test (SIT). Post-infusion serum aldosterone level was 6.99 ng/dL. A diagnosis of primary hyperaldosteronism secondary to right adrenal adenoma was considered. She was started on spironolactone along with prazosin and verapamil. Following spironolactone treatment, her blood pressure normalized. After adequate glycemic and blood pressure control, she underwent laparoscopic right adrenalectomy. The histopathological report of operative specimen was suggestive of adrenal lipoma. There were also foci of adrenocortical hyperplastic nodules in the rest of the gland. Following surgery, her blood pressure and glucose levels were maintained with lesser dose of medications. Postoperative PRA was 1.17 ng/mL/hour, serum aldosterone was 9.93 ng/dL, and ARR was 8.48, which showed biochemical normalization. Conclusion: Although very rare, adrenal lipomas should always be considered when evaluating adrenal tumors. They are usually non-functional and asymptomatic, but can be associated with symptoms related to local tumor per se and hormone hypersecretion. Hence, it is important to subject all these patients for biochemical evaluation before surgery to avoid untoward complications. Whether these tumors are completely non-functional or are associated with hormone hypersecretion, needs to be evaluated. The pathogenesis of hormone hypersecretion, if at all, by these tumors, needs to be looked for. Clinical significance: We present a very rare case of adrenal lipoma with surrounding foci of adrenocortical hyperplastic nodules in the rest of the gland; and probably the first case of its kind in association with primary hyperaldosteronism.