Arteriohepatic dysplasia (AHD) is a multisystem disorder which is characterized by liver, heart, eye, and bony abnormalities. A characteristic facies is also present. The etiology is not clear, and some authors have favored an in utero toxin or infection as the cause. Other reports have shown parent-to-child transmission suggesting an autosomal dominant form of inheritance. This study documents the presence of AHD characteristics in four successive generations of a single kindred. Overall, 15 of 24 members had at least some characteristics of AHD. In addition to the usual findings, renal disease, a small flat face on lateral X-ray, and mild conductive hearing loss were frequently noted in this kindred. Physiological studies on one member revealed a normal bromosulfophthalein Tm and S but a late rise in plasma bromosulfophthalein and indocyanine green. This study strongly supports an autosomal dominant form of transmission for the AHD syndrome. The ease of determining the eye, bone, and heart abnormalities should simplify genetic studies of other patients' families. In addition, because these characteristics are recognizable at birth, documenting their presence in a jaundiced neonate should spare the infant unnecessary and potentially dangerous surgery to rule out extrahepatic obstruction, especially if one or more family members demonstrate the same abnormalities.