A 38-year-old man in a wheelchair for spinal cord injury from a traffic accident was referred to our hospital with complaint of dull right flank pain. The patient had a history of skin ulcer formation on both toes due to poor circulation. Abdominal ultrasonography and computed tomographic scan revealed a huge retroperitoneal tumor compressing the right kidney. Serum levels of cortisol, adrenocorticotropic hormone, aldosterone, adrenalin, noradrenalin, urinary levels of vanillylmandelic acid, homovanillic acid, 17-ketosteroid and 17-hydroxycorticosteroid were all within normal limits. Combining all preoperative diagnostic information, the possibility of retroperitoneal liposarcoma could not be thoroughly ruled out. The patient consented to undergo resection of the tumor with the right kidney. Prior to the scheduled operation simulation of the positioning on the operation table was done to evaluate the compressing pressure. During operation decompressing pads were used to minimize the compressing pressure and intermittent pneumatic compression was also used to prevent pressure ulcer and facilitate circulation to the lower extremities. Five thousands units of heparin (FragminR, Pfizer, USA) was used daily to prevent deep vein thrombosis until the patient could use his wheelchair. Perioperative rehabilitation was conducted by a team of physical therapists. The excised tumor weighed 1,320 g. Pathologic diagnosis was an adrenal myelolipoma. Convalescence was uneventful and the patient was discharged on the 10th post operative day. Between 1992 and 2006, 80 cases of adrenal myelolipoma were reported in Japan. Our case was found to be the second largest one. We reviewed these 80 cases and discussed the diagnosis and treatment strategy of the adrenal myelolipoma. We also discussed the perioperative management for patients with spinal cord injury.