HC is an uncommon--although readily recognizable-clinical entity. Although current hepatobiliary imaging has improved, accurate staging of HC preoperatively is difficult. In patients who have potentially resectable HC, careful preoperative preparation with biliary drainage, portal vein embolization, or both is indicated because major hepatic resection has become an essential component of surgical treatment, and these interventions may reduce perioperative risks. Currently, lobar or extended lobar hepatic and bile duct resection, regional lymphadenectomy, and Roux-en-Y hepaticojejunostomy are the treatments of choice for HC. Whether major vascular resection coupled with these procedures or hepatic transplantation in selected patients will improve overall survival is unknown. Finally, current outcomes dictate investigation of effective adjuvant therapy.