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Adrenal myelolipoma is a rare, benign, non-functioning tumor, composed of mature adipose tissue and hematopoietic cells. We present the case of a 26-year-old woman who presented with abdominal distention and right back pain radiating to the right hypochondrium. Computed tomography of the abdomen revealed a large retroperitoneal mass, which was suspected to be a retroperitoneal liposarcoma. All hormonal studies related to adrenal gland were within normal limits. Open surgery resection was performed. Histopathology showed an adrenal myelolipoma with 39 × 21.5 × 8.5 cm and weighting 4930 g h. The patient evolved with an uneventful postoperative period.