Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is one of the two entities which come under the realm of non-inflammatory cystic lesions of the pancreas, the other one being mucinous cystic neoplasm. The clinco-pathological features of intraductal papillary mucinous neoplasm of the pancreas are unique. The patient had a long history of symptoms suggestive of chronic pancreatitis. Endoscopic retrograde cholangiopancreatography revealed dilatation of the main duct, ultrasonography showed cystic dilatation interpreted as pseudocyts. Current thinking suggests that at best IPMN represents a premalignant condition and, as such, surgical intervention is recommended. The aim should be to resect all gross disease while attempting to achieve a negative surgical margin, which in the majority of cases can be achieved by a partial or subtotal pancreatectomy, which was performed on the patient. Histologically, they demonstrate a spectrum of cellular atypia ranging from minimal mucinous hyperplasia to frank invasive carcinoma. Although the neoplasms are less aggressive as a group than conventional pancreatic ductal adenocarcinoma, patients with IPMNs may pursue a deadly course, even in the absence of identifiable invasive carcinoma thus emphasizing the importance of recognizing IPMNs and initiating optimal modalities of treatment.