Intraductal papillary and mucinous tumors are rare. We retrospectively analysed clinical, surgical and histological features and outcome of 41 operated patients (29 males, 12 females, mean age = 63 years). The commonest presenting manifestation was acute pancreatitis (41%). Tumor was located in only one pancreatic segment in 45% cases. Forty one per cent of patients had invasive carcinoma, 20% had tumor with severe dysplasia and 39% with minimal or moderate dysplasia. Only elevated age was significantly associated with invasive carcinoma. Eleven out of 17 patients with invasive carcinoma (65%) had a recurrence after surgery and 6 (35%) died. Among 24 patients with noninvasive tumor, 2 (8%) recurred without tumor-related death in the follow-up (48 months). This study underlines the need for early surgical resection in patients with intraductal papillary and mucinous tumor because of the high frequency of invasive carcinoma and the poor outcome of patients with invasive carcinoma.