Aim: To present a rare case of congenital polysplenia in adult patient present with sudden abdominal pain due to twisted 
wondering spleen in the pelvis. 
Methods: This is a single case report investigated preoperative and examined clinically where surgical intervention was 
performed due to rapidly progressive enlarged hypogastric mass. 
Result: Adult man with polysplenia and minor congenital heart disease presented with twisted accessory spleen wondering in the pelvis with long pedicle. 
Discussion: Polysplenia syndrome (PSS) is a type of situs ambiguous,usually associated with severe congenital heart 
diseases and rarely these patient reached adult life. The unique sign of the polysplenia is the presence of multiple spleens, ranging from 2 to 16. We present a case of polysplenia in adult patient with minor heart anomalies and four spleens one of them is very huge and discovered as emergency with torsion wondering pelvic spleen.