Abstract: Objective To discuss the clinical diagnosis and treatment of Mirizzi syndrome (MS) type Ⅲ. Methods One case diagnosed with Mirizzi syndrome type Ⅲ in the First Affiliated Hospital of Liaoning Medical University in 2015 was enrolled in this study. The clinical diagnosis and treatment was reported and its related literatures were reviewed. Results A 34 years old man admitted to our hospital with upper abdominal pain on and off for 11 years and sclera jaundice of 5 days. He was initially diagnosed as gallstone and choledocholithiasis in inpatient department according to the liver function, abdominal ultrasound, abdominal CT and Magnetic Resonance Cholangiopancreatography. Surgical treatment plan was laparoscopic cholecystectomy, section of common bile duct, calculus remove and drainage with T tube. However, it formed cholecystobiliary connection unexpectedly (cholecistobiliary fistula) during operation, and the patient was diagnosed as MS type Ⅲ. In consequence, choledochoplasty was performed. Cholangiography was carried out two weeks after operation, and it suggested that the extra-and intra-hepatic biliary were smooth. After removing the T tube, the patient was discharged. Conclusion It suggests that we should be cautious of choosing laparoscopic treatment for patients with highly suspected MS.