We herein present a case of spontaneous rupture of intrahepatic bile duct in a patient with perihilar cholangiocarcinoma, which were successfully treated by curative resection. A 60-year-old male patient with perihilar cholangiocarcinoma was decompressed with single percutaneous transhepatic biliary drainage. Two days after right portal vein embolization, the patient suffered from paralytic ileus with marked abdominal distension. Imaging study revealed that marked fluid collection around the liver and whole abdomen, suggesting intrahepatic bile duct rupture. With abdominal drainage and biliary decompression for 2 weeks, the biliary rupture was controlled. To enhance the safety of right hepatectomy, additional right hepatic vein embolization was performed. The patient underwent routine surgical procedures for right hepatectomy, caudate lobectomy and bile duct resection, and recovered uneventfully and discharged 18 days after surgery. This is the first report of a case of spontaneous rupture of intrahepatic bile duct in a patient with perihilar cholangiocarcinoma.