Abstract Objectives: Although rare, the pancreatic cystic neoplasms are detected with increased frequency lately. The treatment of these modalities however remains controversial. Methods: In this case reports a multifocal Intraductal Papillary Mucinous Neoplasm (IPMN) along with Pancreatic Serous Cystadenoma (PSC) were identified in the pancreatic specimen following a distal pancreatectomy. The results of the histopathology confirmed the existence of an IPMN inside a large PSC. Results: In our case, the patient with this extremely rare type of mixed cystic neoplasm was managed successfully with extensive distal pancreatectomy. Conclusions: The preoperative evaluation of the extent of the pancreatic involvement and the definition of the histologic type of the involved neoplasms before the pancreatic resection is challenging.