Situs Ambiguous with Polysplenia of Macrosomia

Situs ambiguous is a very rare disease with one or less per 10,000 newborns whose organs are not in normal position. It usually has spleen and heart malformations, and if it is accompanied by cardiac malformations, mortality is high. Intestinal malrotation is also seen in 70% of the newborn with situs ambiguous and active treatment is needed when intestinal malrotation are found. We report one case of situs ambiguous with polysplenia in macrosomia in a diabetic mother.