Spontaneous perforation of the left intrahepatic bile duct is extremely rare, especially in adults. Here, we report on a case of a 64-year-old woman who had a complaint of right upper abdominal pain for 10 days, which gradually progressed to entire abdominal pain for 3 days, and was admitted to our hospital. Relevant examinations revealed she had a normal cardiac and lung workup, but an obvious abnormal abdominal computed tomography examination, which revealed an enlarged gallbladder, choledocholithiasis with dilatation of the common bile duct (1.8 cm) and intrahepatic bile duct, and a lot of encapsulated ascites. After being given adequate fluid resuscitation and active preoperative preparation, cholecystectomy and common bile duct exploration and perforation repair operation were then performed. The postoperative course was uneventful, and she was discharged with the T-tube in situ. A choledochoscopy examination at week 6 showed the conditions of the intrahepatic and extrahepatic bile duct were good. For these patients, early diagnosis and surgical treatment are essential for good prognosis. The goal of our surgery is to stop bile leakage, resolve choledocholithiasis and cholangitis, and reconstruct the bile duct.