IntroductionAdrenal myelolipomas are uncommon benign adrenal tumors, which mostly occur unilaterally. We describe a rare case of giant bilateral adrenal myelolipoma mistaken for retroperitoneal liposarcoma.Case presentationA 49‐year‐old man developed fever, left flank pain, and a large mass in his left abdomen. Plain computed tomography showed a 23‐cm mass with fat‐containing components in the retroperitoneal cavity, suggestive of retroperitoneal liposarcoma. A similar 9‐cm mass was observed in the right adrenal gland, suggesting adrenal metastasis. With a clinical diagnosis of retroperitoneal liposarcoma, we administered preoperative radiation therapy and performed a bilateral tumor resection. On postoperative day 9, a surgical site infection occurred, necessitating incisional drainage and the administration of intravenous antibiotics. The histopathological diagnosis was bilateral myelolipoma of the adrenal glands.ConclusionIn cases of bilateral retroperitoneal tumors with a large fat component, adrenal myelolipoma should be considered in the differential diagnosis to avoid overtreatment.