Introduction: Adrenal myelolipoma with extramedullary hematopoiesis (EMH) is an exceedingly rare entity with very few cases reported in the literature. We present a rare case of adrenal myelolipoma with EMH. Case report: A 48-year-old man with the previous history of splenectomy for hereditary spherocytosis was presented with 12 × 12 cm right adrenal tumor and underwent laparoscopic adrenalectomy. The histopathological features confirmed the diagnosis of adrenal myelolipoma with EMH. Conclusion: In any patient with a history of chronic hemolytic anemia and adrenal mass, it is imperative to consider EMH as a possible diagnosis.