Asplenia syndrome is characterized by complex congenital heart defects, asplenia, and abdominal heterotaxy. We report a case of asplenia syndrome associated with cleft lip and alveolus. The patient underwent cheiloplasty while in good condition after cardiac surgery, i. e., Blalock-Taussing shunt operation. Prophylaxis against infective endocarditis is important, and the operation was performed successfully without any infection. There were no circulatory problems, and the patient had an uneventful hospital stay.
