Choledochal cysts are aptly considered a manifestation of a basic abno
rmality of the extrahepatic ductal architecture not limited to the are
as of cystic dilation. The modified classification system of Alonzo-Le
j recognizes five variants by gross morphology. The goal of surgical t
reatment is generally viewed as biliary decompression and excision of
all diseased ductal epithelium. We describe our management of a 22-mon
th-old female with a distinct cystic expansion of the intrapancreatic
common duct as well as proximal biliary dilations. Her surgical treatm
ent consisted of excision of the extrahepatic biliary tree proximal to
the intrapancreatic cystic portion and roux-en-Y portojejunostomy. Th
is case extends the concept of ''choledochal cyst,'' and stresses the
goals of surgical intervention with the importance of follow-up in pat
ients whose particular anatomy prohibits total initial excision.