Pancreatic cysts are relatively rare and commonly seen in adults; in the pediatric age group, pancreatic cysts are very rare. Pancreatic cysts are classified into six types: congenital, retention, duplication, pseudocysts, neoplastic, and parasitic cysts. Clinically and radiologically, it is difficult to differentiate between these types. This may be also difficult pathologically for the first three types, which are also called true cysts. The majority of pancreatic cysts in children are also pseudocysts resulting from trauma, acute pancreatitis, or infection. True congenital pancreatic cysts on the other hand are extremely rare and pose unique surgical challenges. Because preoperative diagnosis of congenital pancreatic cyst is difficult, this condition should be considered in the differential diagnosis of cystic lesions of the pancreas in infants and children.