Following a case report of a newborn with Ivemark's syndrome, we go into etiology, embryology, and anatomy of this congenital cardiovascular disease with asplenia and other visceral abnormalities. The cause of this complex disorder is an exogen or endogen factor active between the 28th and 32nd day of embryological development. Lethality with or without operation is high, therefore clinical diagnosis should possibly be made early by X-ray of chest and abdomen, blood smear, ultrasound, and angiocardiographic investigations.