In 1740, Pohl and Jauch reported an autopsy case of a congenital asplenia in an adult who did not have associated with other anomalies. In 1826, Martin reported a case of congenital asplenia in infant who was combined with congenital cardiac anomalies and the partial transposition of the colon. In 1955, Ivemark reported 14 cases of congenital asplenia which wer associated with cardiac anomalies and situs inversus totalis, visceral symetry, and he susgested that this syndrome should be termed Invemark Syndrome. More than 200 cases were reported since Martins original discription in 1826 throughout the world including 15 cases from Korea. In most cases, this syndrome can be detected before 3 years of age and males are 2 times more frequent than females. Combined anomalies of heart, lung, bowel are more frequent than the single anomly of splenic agenesis only. The prognosis is extremely poor and 70-80% of patients died before 1 years of age. A 5 day-old male infant who died after an operation for generalized peritonitis was found to have congenital asplenia associated with bilaterally tri-lobed lungs, dextroposition of the stomach and the pancrease. We report this case as Ivemark syndrome with the review of the literatures.