Situs inversus is a rare condition which mandates a full understanding of all anatomic relationships prior to invasive procedures. A 76-year-old woman with situs inversus presented with fever and rigors. She had previously undergone endoscopic sphincterotomy and lithotomy for choledocholithiasis, and laparoscopic cholecystectomy for cholecystolithiasis. Laboratory examination revealed hyperbilirubinemia and transaminasimia. Percutaneous transhepatic biliary drainage, percutaneous transhepatic cholangioscopy, percutaneous transhepatic portography, percutaneous transhepatic portal embolization, and visceral angiography were performed without complications. She underwent right hepatic lobectomy, caudate lobectomy and extrahepatic bile duct resection for papillary adenocarcinoma of the proximal bile duct. Full investigation of the anatomical relationships between the biliary tree and the vascular system in the hepatic hilus enabled safe hepatectomy in a patient with situs inversus.