Proteus syndrome was originally described by Cohen and Hayden in 1979. The disorder was named Proteus syndrome by Wiedmann and colleagues in 1983 after Proteus, the giant Greek god of the sea. Proteus syndrome is a rare, sporadic, congenital polymorphic condition. Approximately 200 cases have been reported in the literature, but none has been associated with anal bleeding from hemorrhoids. We describe the case of a 21-year-old man with Proteus syndrome with severe anal bleeding. A hemorrhoidectomy was assumed to be too risky because of the massive venous abnormalities seen on CT. The patient was successfully treated by Doppler-guided haemorrhoidal artery ligation (DG-HAL). Six months after surgery, the patient has had no further episodes of anal bleeding.