Mucobilia is a rare condition, with copious mucus secretion within the extrahepatic bile ducts resulting in obstructive jaundice and cholangitis. Fifteen patients with mucobilia were treated over an 11 year period. Preoperative study included ultrasonography (15), cholangiography (15), liver scanning (2) and computed tomographic (CT) scanning (1). The diagnosis was made preoperatively in 26.6% (4/15) of the patients. The preoperative diagnosis was based on 1) mucinous substance draining out from the papilla after endoscopic cannulation or percutaneous transhepatic biliary drainage fistula 2) multiple amorphous filling defects of the bile duct on cholangiograph 3) biliary duct dilatation with an intrahepatic or liver tumor on image study (sonography or CT scan). The etiology of mucobilia was papillary cholangiocarcinoma in 10 patients, biliary cystadenocarcinoma in three patients, and cystadenocarcinoma of the liver in another two. Intraoperative choledochoscopy revealed an intrahepatic biliary tumor in seven patients, intrahepatic duct stricture or obliteration in six and normal findings in another two. Surgical treatment of the 15 patients included left hepatectomy with choledochotomy in eight and choledochotomy with tumor biopsy in another seven patients. One patient died after T-tube drainage due to septic shock for a 6.6% mortality rate. Postoperative follow-up revealed the continuous drainage of a mucinous substance from the T-tube fistula receiving regular choledochoscopic irrigation in six patients. Four cholangiocarcinoma patients with mucobilia were effectively controlled by external radiation and intraluminal radiation therapy. The overall survival time ranged from 9 months to 11 years 6 months (mean, 46 months). Three patients (20%) are still surviving more than 5 years post diagnosis.