53 yo patient with history of iron deficiency anemia due to celiac disease presented with jaundice and abdominal pain radiating to the back. Upon examination patient was grossly jaundiced, had right upper quadrant tenderness but no palpable mass was felt. Laboratory values were total bilirubin was 11.2 mg/dl, direct bilirubin 7.8 mg/dl, ALT 143 mg/dl, AST 128 mg/dl, Alk. Phos 218 mg/dl, WBC 13.4K with no left shift. Abdominal CT and RUQ ultrasound revealed a subhepatic cystic mass and no gallstone or biliary dilation was noted. ERCP revealed complete obstruction of CBD by external compression 3cm proximal to the ampulla. A sphinterotomy was done and a guidewire was advanced beyond the distorted and obstructed CBD, during advancement the guidewire curled in the choledochal cyst in the opposite direction of the gallbladder. As there was no evidence of perforation diagnosis of type II choledochal cyst was made. A plastic stent positioned above the choledochal cyst. Placement of the stent relieved the external compression of the cyst. Drainage established and patient rapidly improved. Choledochal cyst excision and Roux-en-Y anastomosis was performed electively. There was no evidence of malignancy in the pathology specimen. Discussion: A choledochal cyst is defined as an isolated or combined congenital dilation of the intrahepatic or extrahepatic biliary tree. Todani and colleagues proposed the five types of congenital cysts. Incidence of choledochal cysts is 1 in 13,000 to 15,000 in Western countries. Type II choledochal cyst, a dicerticulum of common bile duct, is the rarest form. Classic presentation triad is obstructive jaundice, right upper quadrant abdominal pain and palpable abdominal mass. Celiac Disease is a genetically-determined chronic inflammatory intestinal disease induced by an environmental precipitant, gluten. Celiac Disease is very common effecting about one in 250 people. Celiac Disease is associated with many autoimmune diseases including IDDM, Autoimmune thyroiditis, ulcerative colitis, autoimmune hepatitis and malignancies especially lymphoma. Considering the rarity of choledochal cyst and frequency of Celiac Disease association is likely of a casual nature, however this is the first case report of type II choledochal cyst in a patient with celiac disease.