A 74-year-old man with obstructive jaundice presented with a thickened distal bile duct wall. A transpapillary forceps biopsy revealed an adenocarcinoma; however, because the tumor image was different from that of a typical cholangiocarcinoma, endoscopic ultrasound-guided fine-needle aspiration was performed on the tumor and enlarged lymph nodes. The tumor cells were positive for synaptophysin and CD56 with a Ki67 labeling index of 95%, and he was diagnosed with small cell neuroendocrine carcinoma. We diagnosed a bile duct tumor with neuroendocrine carcinoma component with lymph node metastasis. Preoperative chemotherapy for neuroendocrine carcinoma was administered because R0 resection was difficult and the risk of postoperative recurrence was high. Three courses of chemotherapy with carboplatin and etoposide resulted in marked tumor shrinkage, and radical resection was performed 3 months after diagnosis. Postoperative pathology revealed adenocarcinoma in the mucosal epithelium and small cell neuroendocrine carcinoma in the submucosa, most of which resolved with chemotherapy. Carboplatin and etoposide were resumed as adjuvant chemotherapy, and 67 months of recurrence-free survival were achieved after surgery.