Idiopathic fibrosing pancreatitis is a rare cause of obstructive jaundice in children and young adults. Only 30 cases have been previously reported. Four children with this entity have been treated at Egleston Children's Hospital at Emory University since 1970. Diagnosis was established by imaging studies and open pancreatic biopsy. Treatment was by bilioenteric bypass, and excellent results have been achieved with follow-up periods up to 25 years.