Ivemark syndrome is a rare disorder of right heterotaxy which affects multiple organ systems of the body as a result of lateralization defects with situs inversus, asplenia or polysplenia due to defective left-right axis development, which is considered as the primary developmental field defect, characterized by cardiac and other organ abnormalities such as midline liver, right sided gastric fundus, inverted duodenal loop, with increased predisposition in males is reported in a female baby. This life threatening condition with severe complex cyanotic congenital heart disease compounded by the increased susceptibility to fatal infections due to immuno-compromise as a result of asplenia is the usual cause of death in early neonatal life. There is however no cure for Ivemark syndrome and management requires a multidisciplinary approach of surgical repair of cardiac malformations, protective immunization, antibiotic prophylaxis with aggressive treatment in event of any infections, may decrease the risk of morbidity and mortality
