Absent pulmonary valve syndrome and L-transposition of the great arteries are rare congenital anomalies. To our knowledge, patients with both have not been previously reported. We present a case of surgical success in the patient with absent pulmonary valve, L-transposition of the great arteries, dextrocardia, double outlet left ventricle, hypoplastic anatomical right ventricle, valvular aortic stenosis and tracheomalacia. We performed anatomical left ventricle to pulmonary artery shunt using an 8-mm expanded polytetrafluoroethylene bicuspid-valved conduit, pulmonary artery plication, and tracheostomy. The patient was discharged in a stable condition while waiting for the Glenn procedure until getting her parent's permission.