Case reports of 55 patients with arteriohepatic dysplasia are evaluated (51 cases reported in the literature, 4 own cases). The main features of this syndrome are dysmorphous facial structure, peripheral pulmonic stenoses, intrahepatic cholestasis, and growth retardation secondary to the liver impairment. In addition, the patients may exhibit vertebral anomalies, retarded mental and sexual development, ocular and renal anomalies. The syndrome probably is inherited as an autosomal dominant trait with variable penetrance and expressivity. Therapeutically cholestyramine in high dosage, phenobarbital and fat-soluble vitamins may be tried.