Retroperitoneal neurilemmomas are the rarest of all retroperitoneal tumors. A rare case of benign retroperitoneal neurilemmoma is presented. Pre-operative diagnosis of these tumors is difficult, and the majority are asymptomatic. However, this patient suffered from a urinary tract infection because of a distortion of the ureter, and the spinal canal was also involved. Surgical intervention was performed under the impression of neurilemmoma.