BACKGROUND: Mirizzi syndrome is a rare complication of prolonged cholelithiasis, characterized by narrowing of the common hepatic duct due to mechanical compression and/or inflammation due to biliary calculus impacted in the infundibula of the gallbladder or in the cystic duct. OBJECTIVES: To describe a series of eight consecutive patients with Mirizzi syndrome, at a single institution, submitted to surgical treatment and to comment on their aspects with emphasis on the diagnosis and treatment. METHODS: Four women and four men, with a mean age of 61.6 years (42 to 82 years), presenting Mirizzi syndrome were operated between 1997 and 2003. The following items were evaluated: clinical presentation, laboratory results, preoperative evaluation, operative findings, presence of choledocholithiasis, type of Mirizzi syndrome according to the classification by Csendes, choice of operative procedures, and complications. RESULTS: The most frequent symptoms were abdominal pain (87.5%) and jaundice (87.5%). All the patients presented altered hepatic function tests. The diagnosis of Mirizzi syndrome was intra-operative in seven (87.5%) patients, and preoperative in one (12.5%). Cholecystocholedochal fistula associated with choledocholithiasis was observed in three (37.5%) cases. Mirizzi syndrome was classified as Csendes type I in five (62.5%) patients, type II in one (12.5%), type III in one (12,5%) and type IV in another (12.5%). Cholecystectomy, as an isolated surgical procedure, was performed in four (50.0%) patients. One (12.5%) patient was submitted to partial cholecystectomy and closure of the fistulous orifice with the central part of the infundibula. Two (25.0%) patients were submitted to cholecystectomy and side-to-side choledochoduodenostomy and another (12.5%) to side-to-side choledochoduodenostomy remaining the gallbladder in situ. Seven (87.5%) patients had an uneventful recovery and were discharged in good conditions. One (12.5%) patient presented a postoperative sepsis due to a sub-hepatic abscess, and was reoperated. There was no operative mortality. CONCLUSION: The preoperative diagnosis of Mirizzi syndrome is difficult and an awarded suspicion is necessary to avoid lesions of the biliary tree. The problem may only become evident during the operation due to firm adherences around Calot's triangle. The success of the treatment is related to a precocious recognition of the condition, even at the time of surgery, and adapting the management considering to the individual characteristics of each case.