BACKGROUND: Mirizzi syndrome is a rare presentation of long-standing cholelithiasis. It occurs when gallstones become impacted in either the gallbladder neck or the cystic duct, causing an obstruction of the common hepatic duct by extrinsic compression. Furthermore, impacted stones may cause pressure necrosis of the adjacent bile duct and produce a cholecystobiliary fistula. Although the definition of this syndrome varies somewhat among authors, Csendes et al. defined four evolving stages of patients with Mirizzi syndrome and cholecystobiliary fistulas. The aim of this study was to observe the clinical characteristics and to review the literature for better management in this clinical situation. METHODS: We retrospectively observed 7 patients who had been diagnosed with Mirizzi syndrome and cholecystobiliary fistulas perioperatively at Hanyang University Hospital. RESULTS: The 6 male patients and the one female patient had an average age of 58 years (range, 39 to 74 years). Jaundice was present in all patients. Six patients complained of abdominal pain, and two patients had acute inflammatory signs, such as fever/chill. Preoperative evaluations suggested Mirizzi syndrome in only two patients. A cholecystectomy was performed in all patients, followed by repair of the common hepatic duct and T-tube choledochostomy in three patients. A hepaticojejunostomy was required for the three difficult patients. The Csendes et al. classification was type I in one patient, type II in four, and type III in two. CONCLUSIONS: Since preoperative diagnosis of Mirizzi syndrome remains difficult, a high index of suspicion is required to diagnosis the condition, and awareness of the cholecystobiliary fistula condition is of the utmost importance for safe and optimal management.