Cystic fibrosis (CF) is the most frequent genetic disease in the Caucasian population. It seems to be associated with an increased risk of digestive cancer but only few cases of pancreatic tumors have been reported. As pancreatic lesions of the pancreas in CF patients are not rare, their etiological diagnosis is substantial. We report herein a case of a mucinous cystadenoma in a patient with CF. Diagnosis and management of pancreatic cystic tumors in patients at high risk of operative morbidity are challenging. When the potential malignancy of a pancreatic cystic mass cannot be well established in a CF patient, partly because of frequent chronic pancreatitis lesions, we suggest that surgical management should be undertaken instead of radiological surveillance.