Although the incidence of gallbladder cancer is relatively low, Korea is one of the countries with the highest incidences of gallbladder cancer in the world. Gallbladder cancer is also often diagnosed in an advanced state, and has poor prognosis. A gallstone is one of the major risk factors for gallbladder cancer and a majority of epidemiological studies support correlation between cholelithiasis and gallbladder cancer. Clear mechanism, however, regarding how gallstones cause gallbladder cancer is not known. In this article, results of studies about the relationship between gallstone and gallbladder cancer were thoroughly reviewed. Also, it was discussed whether prophylactic cholecystectomy is necessary to prevent gallbladder cancer in asymptomatic gallstone patients without other risk factors such as a gallbladder polyp or porcelain gallbladder.