The case of a 66 year old woman admitted with a picture of jaundice acute cholangitis is reported. Ultrasonography showed a dilatation of intrahepatic bile ducts, gallbladder hydrops with several stones, enlarged common bile duct (CBD) with hyperechoic material inside and a cystic tumor with hydatid features. With a strong suspicion of a hydatid cyst ruptured in the biliary tree with biliary obstruction, endoscopic cholangiopancreatography was performed. The diagnosis was confirmed by endoscopic retrograde cholangiopancreatography and the hydatid membranes were extracted from the CBD with subsequent clinical improvement. The second step of treatment comprised the surgical cure of the cyst and cholecystectomy. The data from the literature are finally presented with a special emphasis on the ultrasound diagnosis and the endoscopic treatment.