Ileal adenocarcinoma is rare and poor prognosis. In this report, we present four years recurrent free case of advanced distal ileal adenocarcinoma with 21 retrieved lymph node metastases by treating initial surgery and adjuvant chemotherapy. A 38-year-old man was admitted to our hospital with complaining of abdominal pain, abdominal fullness and subileus due to advanced ileal adenocarcinoma. Laparoscopic assisted illeocolectomy with en bloc removal of regional lymph nodes was performed. Gross examination of resected specimen revealed type 2 tumor in the terminal ileum. On microscopic examination, poorly differentiated adenocarcinoma invaded into subserosa and 21 of the 35 retrieved regional lymph nodes metastases were diagnosed. The patient had no conditions associated with an increased risk for small intestine adenocarcinoma, although the patient was younger than usual cases of small intestine adenocarcinoma. Finally, the patient was diagnosed as primary sporadic poorly differentiated adenocarcinoma of ileum with multiple regional lymph nodes metastases (T3N2M0). He had received adjuvant chemotherapy after operation with SOX + bevacizumab agent for 6 months and followed oral administration of S-1 for 12 months. The patient remained well with recurrence free for four years following the initial surgery.