A 7-month-old female infant with paroxysmal dyspnea and cyanosis of the limbs was diagnosed with anomalous right pulmonary venous drainage (Scimitar syndrome) by echocardiography. Wet rales were heard in both lungs by physical examination. She was admitted into our hospital with a preliminary diagnosis of “Scimitar syndrome and lung infection.” During the symptomatic treatment in the medical ward, respiratory distress and progressive worsening of cyanosis occurred and caused secondary congestive heart failure and respiratory failure. Emergency surgery was required to correct the anomalous pulmonary venous drainage. Once multiple pulmonary veins were affected by Scimitar syndrome and accompanied by congenital pulmonary dysplasia, the risk of heart and lung failure was significantly increased. Pulmonary vein obstruction may be life-threatening in a small number of children. Early surgery is the key to reversing this condition. Anesthetic management of these children should focus on respiratory management, regulating pulmonary vascular resistance, aggressive cardiac support, and treating pulmonary hypertension.