Introduction: Mediastinal parathyroid adenomas (MPAs) result from aberrant migration of the parathyroid during development, constituting 20% of ectopically located adenomas. Aortopulmonary window (APW) parathyroid adenomas are rare, accounting for only 1% of ectopic mediastinal adenomas and 0.24% of all parathyroid adenomas. We present here a case of an MPA in the APW which was managed surgically and a brief review of the literature. Case description: A man in his 30s presented with low-backaches and recurrent renal calculi for 7 years, and also a history of fatigue, increased irritability, and insomnia. He was detected to have hypercalcemia, with inappropriately elevated parathyroid hormone (PTH), with background chronic kidney disease probably due to nephrocalcinosis. The ultrasound thyroid showed small subcentimetric doubtful parathyroid lesions in their ectopic locations. Tc99SestaMIBI scan detected an MPA, which on CT neck and thorax revealed an ~ 2.1 × 2 cm lesion in the APW. He underwent a left posterolateral thoracotomy with excision of APW parathyroid + cervical exploration and excision of left superior parathyroid and left inferior parathyroid biopsy. His PTH and calcium levels normalized postoperatively. Discussion: Aortopulmonary window parathyroid adenomas are very rare, usually located in the middle mediastinum, and are postulated to be supernumerary glands in 58–60%. The blood supply is derived from the mediastinum, from the bronchial arteries or internal mammary artery. The cervical approach is not feasible for adenomas in this location, and video-assisted thoracoscopic surgery (VATS)/thoracotomy is required. Conclusion: Structural imaging is paramount when MPAs are suspected, for appropriate surgical planning and management.