Surgical resection for distal cholangiocarcinoma is usually carried out using pancreaticoduodenectomy (PD). However, because PD is a complex procedure with a high rate of postoperative complications, the surgical indications should be carefully considered, especially for patients with a decreased performance status, significant comorbidities, and/or anatomical anomalies. If curatively carried out, a less invasive, local resection may be an alternative procedure for such patients. In the current study, we present pancreas-preserving resection of the lower biliary tract in a patient with early-stage distal cholangiocarcinoma. This procedure was selected to avoid PD with arterial reconstruction because of arterial anomalies. After an abdominal exploration, a cholecystectomy was carried out and the common hepatic duct was transected. The bile duct was dissected from the pancreatic parenchyma without pancreatic resection, downward to the biliopancreatic ductal confluence. Next, a duodenotomy was done opposite Vater's ampulla. The duodenal mucosa around Vater's ampulla was incised and dissected, and the main pancreatic duct (MPD) was divided. The bile duct was completely separated from the pancreatic parenchyma, and the lower biliary tract was totally "cored-out". After resection, the MPD was re-implanted into the duodenal wall, and the duodenotomy was closed. Finally, a Roux-en-Y hepaticojejunostomy was created. Postoperative course was uneventful. No tumor recurrence has been observed for 21 months after the operation. Thus, pancreas-preserving resection of the lower biliary tract appeared to be appropriate for our patient. This organ-preserving approach can be a useful, alternative procedure in selected patients.