Background and aim: Adrenal myelolipoma is a benign tumor composed of both lipomatous and myeloid components. It is usually asymptomatic in presentation. Another spectrum may present with rupture and hemorrhage. Usually, myelolipoma are non-functional but an endocrine abnormality is seen in 7% of cases. Radiologically diagnosed and asymptomatic adrenal myelolipomas can be kept on follow-up. Symptomatic cases need adrenalectomy. Adrenal Histoplasmosis usually occurs as a part of disseminated histoplasmosis. This is a fungal infection which seen in both immunosuppressed and immunocompetent individuals. Here by reporting a patient presented with Unilateral adrenal myelolipoma with minimal autonomous cortisol excess (MACE) and disseminated histoplasmosis. Case description: This is a case report of a 64-year-old gentleman presented with vague abdominal discomfort and evaluation found to have large unilateral Adrenal myelolipoma with MACE. The patient underwent Laparoscopic adrenalectomy. Histopathological examination showed histoplasmosis, and was managed by antifungals. Conclusion: Symptomatic adrenal myelolipoma is managed by adrenalectomy. Prompt antifungal treatment has avoided complications of disseminated histoplasmosis. Clinical significance: This type of disseminated histoplasmosis in unilateral myelolipoma with MACE is a rare presentation and worth reporting.