A 17-years-old man with the juvenile form of MLD developed massive hemobilia. CT and US scans showed blood and clots filling the gallbladder and the biliary ways, with no bleeding source seen at selective angiography. Explorative laparotomy evidenced bleeding from a papillomatous gall bladder mucosa, resolved with cholecystectomy. Histologic examination with specific colorations diagnosed hemorrhagic cholecystitis from metachromatic leukodystrophy of the gallbladder. This is, to our knowing, the third case reported in Literature, and thus hemorrhagic cholecystitis may be considered a life-threatening complication of MLD to be prevented with cholecystectomy as soon as signs of gallbladder pathology (papillomatosis/polyposis, jaundice, abdominal pain) are suspected.