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Dilatation of the main pancreatic duct (MPD) ˃50 mm is a rare phenomenon. We present the case of successful surgical treatment a 63-year-old female patient who presented epigastric pain and weight loss of 10 kg in 6 months. Based on preoperative examination, the dilatation of MPD was 75 mm. The preliminary diagnosis was main duct intraductal papillary mucinous neoplasm. Extended pylorus-preserving pancreatoduodenectomy with resection of the body of the pancreas was performed. According to the results of pathological and immunohistochemical studies, performed by independent experts, the final diagnosis of congenital anomaly of the ductal system and neuroendocrine tumor of the accessory pancreatic duct was made. [ABSTRACT FROM AUTHOR]