A 15-year-old girl evaluated for short stature and delayed puberty was found to have features of syndromatic arteriohepatic dysplasia or Alagille's syndrome. The characteristic triangular face, stubby nose, peripheral pulmonic stenosis, a history of prolonged neonatal jaundice and evidence of hepatic parenchymal disease were present as well as bilateral small kidneys and delayed puberty. The recognition of kidney involvement in this syndrome and the characterization of the frequency and nature of the renal disorder are of great prognostic significance and could influence the morbidity and mortality of these patients.