Jarcho-Levin syndrome is a rare central skeletal system defect with the involvement of ribs and vertebrae and association of short trunk stature and some other organ anomalies. One of the common problems is different rate of respiratory abnormalities. Chest X-Ray is the primary base for the diagnosis, but spiral 3D CT scan give us more accurate evaluation of the ribs situation. There are also cardiac problems associated with this syndrome. In this case we present a 9- month- old infant who had Jarcho-Levin syndrome with right-sided heart failure.