A case of a complex fetal heart malformation with left isomerism, a "right sided" double outlet ventricle, and aortic isthmus stenosis combined with asplenia was diagnosed by ultrasonography at 26 weeks' gestation. The pregnancy was terminated at the request of the parents in the 27th week of gestation. Because the prognosis for a fetus with cardiosplenic syndromes depends mainly on the severity of the cardiac abnormality cases of visceral heterotaxy should be classified according to the predominant feature, the heart malformation.