Congenital arteriovenous malformation (AVM) in the male pelvis is extremely rare. We present a case of this disease with massive bladder bleeding which caused severe hemorrhagic shock. Diagnosis of pelvic AVM was confirmed by computed tomography and angiography. He was treated with transcatheter arterial embolization of the bilateral internal iliac arteries. We discuss the clinical course and the treatment for AVM in this case, and review the literature. # ja.