We reviewed 37 cases described previously in the literature to provide a patient operated upon for a retroperitoneal nonchromaffin paraganglioma with qualified advice concerning followup examinations and prognosis. This type of tumor apparently grows slowly and metastasizes late. A radical operation is the treatment of choice and provides a good prognosis. We suggested that our patient undergo clinical followup and ultrasound investigations at 3 to 6-month intervals.