Introduction: Situs inversus totalis (SIT) is a rare congenital anomaly in which thoracic and abdominal organs are mirrored from normal positions. Hepatolithiasis, common in East and Southeast Asia, often requires hepatectomy for localized disease with parenchymal atrophy. Laparoscopic approaches in SIT patients are challenging due to reversed anatomy and ergonomics. This report presents the first case of laparoscopic anatomical left hepatectomy for hepatolithiasis in a SIT patient, guided by the dilated bile duct and middle hepatic vein. Materials and Surgical Technique: A 57-year-old woman with SIT presented with recurrent cholangitis. Imaging showed multiple intrahepatic stones, strictures, and severe left liver atrophy, indicating localized disease. Laparoscopic left hepatectomy was performed with adaptations: mirrored trocar placement, French positioning, and dissection using the dilated bile duct and middle hepatic vein as anatomical landmarks. The procedure ensured safe navigation of mirror-image anatomy. Discussion: The surgery was completed with minimal blood loss and no complications; the patient was discharged on postoperative day six and remained asymptomatic at follow-up. This case highlights that meticulous preoperative planning, ergonomic adjustments, and bile duct–oriented resection enable safe laparoscopic hepatectomy in SIT, achieving complete stone clearance and reducing recurrence risk in complex hepatolithiasis scenarios.