Objective To investigate the clinical characteristics of patients with Mirizzi syndrome and summarize our experience in diagnosis, treatment and outcomes.Methods The clinical data for all 16 cases of Mirizzi syndrome that were treated at our hospital between 1997 and 2011 were retrospectively analyzed.Results The rate of definitive diagnosis prior to surgical intervention was poor.The surgical procedures used on these patients varied according to Mirizzi syndrome type.Patients with type I Mirizzi syndrome underwent cholecystectomy or subtotal cholecystectomy.Patients with type Ⅱ underwent cholecystectomy followed by closure of the bile duct plus T-tube drainage of common bile duct.Patients with type Ⅲ and type Ⅳ underwent cholecystectomy and hepaticojejunostomy.Treatment of Mirizzi-associated gallbladder cancer was carried out by radical surgical excision of the biliary cystadenocarcinoma.Conclusion The poor rate of preoperative definitive diagnosis of Mirizzi syndrome may contribute to progression to cancer.The operative strategy should be designed according to the various types of Mirizzi syndrome.In order to inhibit progression to cancer or initiate cancer therapy as early as possible, pathological examination should be carried out during the initial operation or immediately afterwards.