Adrenal myelolipoma is a benign, non-secreting tumor composed of adipose and myeloid tissue that can be complicated by retroperitoneal pain or hemorrhage. Its diagnosis is carried by the computed tomography (CT) and confirmed histologically. Surgery may be necessary in case of large volume, symptomatic mass or complication. We report the case of a 36-year-old patient who has a combination of myelolipoma and hereditary spherocytosis, which is extremely rare in the world literature. Computed tomography guided the diagnosis and surgical excision was performed because of the volume of the mass and its symptomatic nature. The evolution at two years was without recurrences.
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