Adrenal myelolipomas are rare benign tumors. Most of the cases are asymptomatic and discovered incidentally. We are reporting a case of myelolipoma involving right adrenal cortex of a 40-year-old woman who presented with abdominal pain. A short review of etiology, clinical features, and differential diagnoses of this neoplasm are also discussed. Radiologic features are often helpful in diagnosis but histology must be done to exclude other fat-containing lesions. Although uncommon, myelolipomas should be considered in differential diagnosis of retroperitoneal lesions.