A 16-year-old boy with congenital hepatic fibrosis, on whom a portocaval anastomosis was performed because of bleeding esophageal varices, developed lip cyanosis, exertional dyspnea, and clubbing of the fingers 7 years postoperatively. This progressive syndrome was caused by a right-to-left shunt due to the presence of pulmonary arteriovenous fistulas. Increased erythrocyte sedimentation rate, hypergammaglobulinemia, and histological features of cholangitis were also present. It is proposed that this unusual pulmonary complication of congenital hepatic fibrosis could be due either to congenital pulmonary abnormalities or to a chronic unrecognized cholangitis causing a progressive deterioration of liver histology and function, with subsequent opening of pulmonary arteriovenous shunts.