A 13-month-old girl with cyanotic congenital heart disease; single atrium, single ventricle, common atrioventricular (AV) valve, pulmonary atresia and total anomalous pulmonary venous drainage, suspected of asplenia underwent ear tube surgery for otitis media. She had undergone bilateral Blalock-Taussig shunts for her heart disease. She had congestive heart failure due to moderate to severe common AV valve regurgitation and often experienced respiratory tract infection with sputum. Oxyhemoglobin saturation measured by pulse oximetry was 75-80% and polycythemia was found in complete blood count. We chose tracheal intubation for her airway management because of a large amount of sputum. General anesthesia was maintained with sevoflurane, nitrous oxide and oxygen for ear tube surgery. During anesthesia she showed several episodes of desaturation which were well managed by frequent tracheal suctioning. Her circulation was stable with 50% N2O and sevoflurane 1.7-2.0%. The operation was performed uneventfully and the patient was discharged to the ward after tracheal extubation. Asplenia is frequently complicated with cyanotic congenital heart disease and increased susceptibility to bacterial infection. Anesthesia for these patients with upper respiratory infection should be managed with tracheal intubation even for a short surgery.