Mirizzi syndrome is a rare condition caused by the obstruction of the common bile duct or common hepatic duct by external compression from multiple impacted gallstones or a single large impacted gallstone in Hartman's pouch. The condition can easily be confused with choledocholithiasis, bile duct stricture or cholangiocarcinoma due to the presence of obstructive jaundice hence may be overlooked due to the rarity of the condition. The incidence of Mirizzi syndrome among patients with gallstones is reported to range from 0.63 to 5.7%. Furthermore, it poses a differential diagnosis dilemma for the physician as well as radiologists because there are no clinical features or diagnostic procedures that have a 100% specificity and sensitivity. Laparotomy is the preferred surgical technique of choice. For the patients who are poor surgical candidate, mainstay of treatment is biliary stent placement for the restoration of normal biliary drainage. Due to low incidence of the Mirizzi syndrome, an elevated index of suspicion is required to diagnose this condition. At present, there are no well-developed, internationally recognized clinical guidelines for the management of this syndrome. Furthermore, the diagnostic procedures available still pose a barrier in the ability to confirm the diagnosis prior to surgical treatment, even though the diagnostic rate has increased dramatically.