A 76-year-old man came to our hospital for a close examination after an abnormal finding during a medical checkup. Upper gastrointestinal endoscopy revealed a circumferential flat lesion with irregularity in the second to third portions of the duodenum. Biopsy diagnosed papillary adenocarcinoma. Contrast-enhanced CT of the abdomen showed no evidence of lymph node enlargement and distant metastasis. Endoscopic depth of the lesion was estimated to be intramucosal carcinoma, but it was approximately 60 mm in size, circumferential, and located near the papilla Vater. Therefore, endoscopic resection was deemed difficult. Subtotal stomach-preserving pancreaticoduodenectomy was performed. Postoperative pathological examination revealed type 0-Ⅱa, tub1>pap, pTis, Ly0, V0, 80×50 mm, BD1, Ex0, Pn0, pPM0, pDM0, pN0, pStage 0. There has been no recurrence since then. Lateral spreading duodenal carcinoma is a rare disease, and endoscopic resection, local resection, and pancreaticoduodenectomy have been reported as treatment options. We report a case of resection of a large lateral spreading duodenal carcinoma with a review of the literature.