We report a case of spontaneous rupture of the common iliac artery associated with fibromuscular dysplasia (FMD). A 21-year-old previously healthy male presented with acute onset of colic pain, suspected to be caused by a ureteral stone. Abdominal computed tomography and angiography revealed a retroperitoneal hematoma caused by rupture of the common iliac artery. In spite of an emergency operation initiated quickly, the patient died. A pathological examination demonstrated FMD of the common iliac artery. Although very rare, it is important to bear in mind that the possibility of retroperitoneal hemorrhage exists in patient with sudden lumbago.