Hemolytic anaemia often challenges congenital heart surgery. Hereditary
spherocytosis is a rare familial hemolytic anaemia. When associated with
congenital heart disease, the safe performance of cardiopulmonary bypass
becomes a priority. The increased risk of hemolysis during
cardiopulmonary bypass could potentially lead to significant secondary
organ damage. Till now, only very few reports of successful repair of a
congenital heart defect in patients with hereditary spherocytosis have
been reported. We report the only case of successful repair of a
congenital heart defect in an infant with hereditary spherocytosis.