Abstract: Objective　To explore the diagnosis and treatment of the the aortopulmonary window (APW) combined with anomalous origin of the right pulmonary artery from the aorta (AORPA) and interruption of the aortic arch (IAA) in children. Methods　The clinical data of one pediatric patient diagnosed of the APW combined with AORPA and IAA in April 2013 was retrospectively analyzed. Results　The 4-month-old female patient was diagnosed with ventricular septal defect in local hospital. The conservative treatment was not effective and the ventilator weaning was not successful. After admission, the echocardiography suggested APW, AORPA, patent ductus arteriosus and severe pulmonary hypertension. The oliguria and the big difference of arterial pressure between upper and lower limbs were presented after surgery. A computed tomography scan showed that the patient also had IAA. After a second surgery, the patient recovered and was discharged. During 28 months of the follow-up, the growth and development of the patient were the same as normal children. Conclusions　APW combined with IAA and AORPA require early surgical procedures after diagnosis. Angiocardiography and other tests should be performed to avoid miss-diagnosis before surgery.