|Polysplenia syndrome is a rare disease with presence of multiple spleens in many different sizes and numbers, involving multiple congenital anomalies, such as cardiovascular anomalies, abdominal heterotaxia, major venous system and bronchial malformation. The spectrum ofabnormalities in patients with this syndrome is broad. It often occurs in childhood, only 25% of patients diagnosed with polysplenia survive past 5 years of age and only 10% survive into mid-adolescence because of associated anomalies. Severe cardiac malformations, biliary atresia, and duodenal atresia are the common causeds of death during infancy. Some patients with polysplenia syndrome have a normal heart or only minor cardiac defects, are often diagnosed incidentally in patients being treated for other disease. A 20 year old man presented with intermittent febrile sensation and cough. He was diagnosed with duodenal atresia shortly after birth, and had surgery. During the management of pneumonia, he complained of abdominal discomfort. An abdominal computed tomography(CT) showed multiple spleens, intestinal malrotation, hypoplasia of pancreas tail portion, and absence of the suprarenal inferior vena cava(IVC) with continuation of azygos vein. These findings are consistent with criteria of polysplenia syndrome. In Korea, few cases have been reported in adults and our case showed the incidental finding of polysplenia syndrome while examining the patient who comoplaining of abdominal discomfort and cough.