Reported in the present study are the clinical and autopsy finding of a male of 13 years and 6 months old having a highly advanced cardiac deformity with some considerations on literatures. 1) Clinical findings: The height and the weight of this patient were equivalent to those of a boy of 11 years old showing a marked growth retardation. The patient showed drumstick fingers, cyanosis, dyspnea and crouching phenomenon. 2) Laboratory findings: Electric cardiograms showed waves difficult to explain, and X-ray pictures showed characteristic two-chambered heart. 3) Autopsy findings: Macroscopically, the patient was found to have two-chambered heart, asplenia and multi-lobular lungs.