We report a case of a neonate who was diagnosed as having congenital leukemia after presenting with an intracranial hemorrhage. The chief symptom was early-onset jaundice due to the hemorrhage. The intracranial hemorrhage and post-hemorrhage hydrocephalus advanced. In addition, the leukemia worsened leading to death at 14 days old. The possibility of leukemia, although rare, should be considered as a cause of intracranial hemorrhage in term babies.