Total pancreatectomy has occasionally been performed to treat patients with multiple lesions (such as intraductal papillary mucinous neoplasm (IPMN)) or patients who have undergone repeated pancreatic resection. However, deficiencies of the exocrine and endocrine functions worsen patients’ quality of life. Recently, there have been several case reports citing middle segment-preserving pancreatectomy (MSPP) as a safe procedure and beneficial with respect to preservation of the exocrine and endocrine functions. We herein report the case of a patient who underwent MSPP for repeat pancreatectomy for IPMN and in whom a favorable outcome was achieved. The patient, a 70-year-old man, was diagnosed with branch duct-type IPMN (BD-IPMN) with worrisome features in the pancreatic head and a single cyst in the pancreatic tail, during a preoperative examination of early gastric cancer. Pancreatoduodenectomy was performed for BD-IPMN in the pancreatic head and gastric cancer. A histopathological examination showed an intraductal papillary mucinous adenoma (IPMA) with mild-moderate atypia. During the follow-up, the size of the cystic lesion in the pancreatic tail and the diameter of the main pancreatic duct were gradually increasing. Therefore, at 2 years and 6 months after surgery, distal pancreatectomy with preservation of the spleen (namely MSPP) was performed. The pancreatic resection margin was histologically negative. The length and volume of the remnant pancreas were approximately 6 cm and 10 ml, respectively. A histopathological examination showed an IPMA. The patient had no diarrhea or weight loss without digestive enzymes and maintained favorable glucose tolerance without oral hypoglycemic agents or insulin. He has showed no evidence of new lesions in the remnant pancreas at 3 years of follow-up after the last surgery.