A 29-yr-old man with cystic fibrosis had a 4-yr history of recurrent episodes of obstructive jaundice. Endoscopic retrograde cholangiography revealed a common bile duct smoothly narrowed in its intrapancreatic portion, with dilatation above. A choledochojejunostomy was performed, but the patient died of septic complications. At autopsy, extensive pancreatic fibrosis caused compression of the distal bile duct in a manner which is analogous to that seen in chronic pancreatitis. This complication of cystic fibrosis, not previously reported, may become more prevalent as more patients with cystic fibrosis are living into adulthood.