A 74-year-old man was admitted for evaluation of a pancreatic tumor. Abdominal CT revealed a multilocular cystic tumor with thickened septal walls and an enhanced mural nodule in the head of the pancreas, as well as enlarged para-aortic lymph nodes. FDG-PET revealed FDG accumulation in the nodule and in the para-aortic nodes. The tumor was diagnosed as intraductal papillary mucinous carcinoma (IPMC) with para-aortic lymph node metastases. After 2 courses of chemotherapy with gemcitabine and nab-paclitaxel, the tumor decreased slightly in size, but the lymph nodes did not change. Surgery was then performed. Intraoperative pathology examination confirmed that the para-aortic lymph nodes had only inflammatory swelling. Accordingly, pylorus-preserving pancreaticoduodenectomy was performed. Histopathology examination revealed atypical cells without invasion, and IPMC (TisN0M0;stage 0) was diagnosed. It is thus important for selection of the appropriate treatment approach to determine if enlarged para-aortic lymph nodes are benign or malignant.