A new case of congenital cyst of the pancreas is reported. A 34-year-old wo
man was admitted with a painless, large, epigastric mass. Ultrasonography r
evealed an anechogenic retrogastric tumor. Computed tomography scan describ
ed a liquid cyst of the pancreas which was 15cm in diameter. During surgery
, a well-delimited translucent cyst was found and no local malignancy was o
bserved. Extensive distal pancreatic resection with preservation of the spl
een was performed and a thin part of cephalic pancreas was preserved. The l
iquid of the cyst did not contain any mucus. Microscopic study of the cyst
wall described normal cuboidal cells and congenital cyst of the pancreas wa
s diagnosed. Several diagnoses including hydatid cyst, pseudocyst and cysti
c tumors of the pancreas are discussed. Before surgery, lack of acute pancr
eatitis in recent medical history rules out pseudocyst and hydatid cyst. Du
ring the operation, if cystadenocarcinoma is easily ruled out, macrocystic
serous cystadenoma is more difficult to exclude. Only histological examinat
ion of the cystic wall confirms the difference between cystadenoma and cong
enital cyst which remains an exceptional entity.