When a cystic lesion of the pancreas is encountered, the first priority is to differentiate whether it is an inflammatory or neoplastic cyst. Failure to recognize the true nature of a neoplastic cyst will lead to an incorrect treatment strategy. This was dramatically proven in one of our patients. Nevertheless, because of the slow growth of this type of tumor, its tendency not to infiltrate adjacent structures and the relatively low likelihood of metastasis, operative resection should be attempted in all patients, even in more advanced stages.