This case was a stillborn male infant to 26 years old multiparous mother, who was born after 41 weeks of gestation. During this gestational period, the mother was careful and uneventful. Antenatal roentgenogram of simple abdomen revealed severe hydrocephalic appearance and craniotomy followed by induced vaginal delivery was performed. This infant was 3.1㎏ in weight and 50㎝ in height, showing enlarged head with increased occipitofrontal length of 15.0㎝ There were no anomalies in face, extremities and others on external examination. In internal examination, a typical alobar holotelenceptaly was observed without other extracranial anomalies except for Meckel's diverticulum. In the past history of the mother, she delivered first baby, male, 2 years ago. The baby was dead within several hours after birth and was anencephaly with mongoloid feature and short extremities. Reviewing of literatures and pathogenesis of the anomaly have been discussed.