Mirizzi syndrome is a form of obstructive jaundice caused by a stone impacted in the gallbladder neck or the cystic duct that impinges on the common hepatic duct with or without a cholecystocholedochal fistula. This syndrome is a rare complication of cholelithiasis that accounts for 0.1% of all patients with gallstone disease. Preoperative recognition is necessary to prevent injury to the common duct during surgery. We present a patient with a preoperative diagnosis of type I Mirizzi syndrome that was confirmed and drained by endoscopic retrograde cholangiography (ERC), followed by subtotal cholecystectomy. A review of the literature covering its clinical presentation, diagnosis, and surgical treatment is also presented.