Polysplenia Syndrome is a rare congenital anomaly accompanied by abdominal, cardiac, vascular and thorax abnormalities. This condition is commonly diagnosed after radiological studies due to childhood cardiac complaints. In this study, a 6-year-old boy patient with tricuspid valve failure, dextrocardia, polysplenia, widening vena azygos and absence of vena cava inferior, pancreas corpus and tail cut is presented. If the anomalies in this syndrome are identified then it will be possible to avoid an understanding as a mass of short pancreas or spleens.