Choledochocele is an extremely rare congenital lesion of the biliary tree causing abdominal pain, pancreatitis, and obstructive cholestasis. Traditionally the therapy for this malformation has been surgery. Recently endoscopic therapy has been utilized alternatively for the treatment of choledocele in adults. We report the case of a 2-year-old girl with a choledochocele who was treated by endoscopic sphincterotomy and placement of a biliary stent. The prosthesis was removed after 4 months. After a follow-up of 20 months the patient remains free of symptoms. Our experience suggests that endoscopic treatment of congenital biliary disease can be performed accurately. Further studies will be necessary to confirm the value of stent implantation in congenital bile duct stenosis.