True left-sided gallbladder (LSGB) is a rare congenital anomaly with an incidence of 0.1–1.2%. It is more common in women than in men at a ratio of 5:1. Its etiology is unknown, although several theories have been proposed. The symptoms are similar to those of a normal gallbladder, which makes the preoperative diagnosis difficult despite imaging studies. In most cases, the diagnosis is achieved intraoperatively. LSGB is associated with biliary anomalies, and the incidence of biliary tract lesions is high. Four-port laparoscopic cholecystectomy is a safe procedure for LSGB. Surgical technique varies according to each case. Preoperative imaging studies have low sensitivity for diagnosis, and the surgeon should be familiar with ductal and vascular anatomical variants in addition to possessing the ability to change the conventional surgical technique to optimize the exposure and minimize the risk of bile duct injury.