Mirizzi syndrome is a rare but serious complication of the gallstone disease. In 1948 Pablo Luis Mirizzi for the first time described the so called ‘functional hepatic syndrome in patients ‘, who had obstructing concretion in d. cysticus causing cholestasis in the general bile duct by external compression. The compression, process of inflammation and wall ischemia result in erosion of the tissue engaged and appearance of various complications – abscess of the gallbladder, perivesicalar abscess, cholecystoduodenal fistula, stricture of bile ducts, biliary fistula. Mirizzi syndrome is still a challenge before the surgeon because of the a change in the normal anatomy and the severe process of inflammation. Good knowledge of the problem is essential for the accurate diagnosis and treatment. The operative strategy has two main goals – provision of efficient passage of the gall and reliable restoration of the hepato-choledochus. Choosing the surgical tactics during the operative intervention is of foremost importance – cholecystectomy in type I and restoration of the integrity of the bile ducts by T-tube drainage, plastic surgery of the hepato-choledochus or bilio-digestive anastomoses in type II-IV. The good knowledge of the problem and anatomic variations keeps safe the surgical team form iatrogenic lesions of the bile ducts and contributes for favourable postoperative results.