Adrenal myelolipomas (AMLs) are rare benign adrenal tumours containing adipose and hematopoietic tissue as a result of reticuloendothelial cell metaplasia. In this case report, we describe the diagnostic evaluation and the operative management of a giant adrenal myelolipoma in a 14-year-old male who has sickle cell anaemia. He presented with a one-week history of bilateral leg swelling. A physical examination revealed a mass in the right lumbar region. Ultrasound of the abdomen revealed a well-defined rounded echo-complex encapsulated supra-renal mass impinging on the upper pole of the right kidney, displacing it downwards. There was also marked compression of the inferior vena cava. A computed tomography scan showed a large mass occupying the right adrenal gland. The patient had an exploratory laparotomy with excision of the right adrenal tumour. Histopathological (Immunohistochemistry) evaluation of the mass confirmed the diagnosis of adrenal myelolipoma. The diagnosis of an adrenal myelolipoma requires a good history, physical examination, a high index of suspicion, imaging techniques, and a thorough histopathological evaluation. Surgery is the main treatment modality and good post-operative management minimizes complications and guarantees rapid recovery.