A 51-year-old male patient visited our hospital with a complaint of anal dysfunction and anal pain. Colonoscopy revealed a lower rectal cancer just above the dentate line, and its biopsy showed endocrine cell carcinoma. Enhanced computed tomography( CT) showed pararectal and right inguinal lymph node metastasis. Leucovorin, 5-fluorouracil, and oxaliplatin (FOLFOX) therapy was initiated first; then, after K-RAS was found to be wild type, 6 courses of FOLFOX plus panitumumab were administered. After the chemotherapy, the main tumor and the lymph node swelling reduced evidently. Its effect was judged as a partial response (PR). Laparoscopic assisted intersphincteric resection, right inguinal lymph node dissection, and temporary ileostomy were performed. The pathology revealed endocrine cell carcinoma (MP, ly2, v2, n0, stage II). Its pathological effect was judged as Grade 1b. Five months after the surgery, the ileostomy was closed.