Polysplenia syndrome is a congenital anomaly in which cardiac, vascular, and thoracic anomalies are seen together. The diagnosis is mostly made in the childhood with radiological examinations which are performed due to cardiac complaints. On direkt X-ray of our patient, dextrocardia was detected. On computed tomography scans, multiple spleens biggest with 5 cm diameter were seen in the left upper quadrant, and the tail of the pancreas was missing. Inferior vena cava was absent and the hepatic veins were directly opening into the right atrium. The azygos vein was dilated and draining into the left sided superior vena cava. The minor fissure was absent in the right lung and the bi-lobed right lung was evaluated as thoracic isomerism. Here we report a rare case of polysplenia syndrome in a seven years old male patient who admitted to our hospital with complaints of shortness of breath and difficulty during running and was diagnosed after physical and radiological examinations.