Objective To summarize surgical experience of eight patients with pentalogy of Cantrell. Methods Six male and two female patients with pentalogy of Cantrell,aged from 4 months to 26 years old, average 7.35 years old, underwent surgical therapy for intracardiac anomalies and extracardiac anomalies from July 2007 to June 2009. Eight case with intracardiac anomalies include one case with only VSD, one case with only ASD, two cases with DORV, four cases with VSD and ASD or PTO. Experts majoring in cardiovascular surgery cooperated with doctors majoring in thoracic surgery and general surgery for satisfactory correction of intracardiac anomalies and extracardiac anomalies and repositioning heart to thoracic cavity. Results Ectopic heart of the first patient was simply repositioned into thoracic cavity following surgery of double outlet of right ventricle in another hospital two years before. Correction of introcardiac anomaly and reposition of ectopic heart finished at one time in 7 cases. Eight patients got full recovery except that residual shunt occurred in the second case which also got full recovery after transcatheter therapy. Ventricular diverticulum was removed in the fourth case because of difficult reposition of ectopic heart.With the help of general surgeon and thoracic surgeon, partial coronary ligament of liver and falciform ligament of liver in the left was cut in the first case and the left half lobe of liver was pushed downward. Bilateral pleural and marginal costal costochondral was cut and make thoracic wall upward so that ectopic heart can reset into thoracic cavity. And then, defect of diaphragm and abdominal wall were repaired with Proceed patch. In the other seven cases, bilateral pericardium and mediastinal pleura was cut and the 7th and 8th cartilage was transected and bilateral costal arch was closed so for complete thoracic angioplasty.Left ventricular dysfunction occurred in the fifth case with DORV and also got full recovery after symptomatic treatment. Full recovery was got in all cases after followingup from 1 to 23 months. No adverse complications occurred and every case live a wonderful life. Conclusion Pentalogy of Cantrell can be cured at one time by accurate correction of cardiac anomalies, cutting of bilateral pleural and marginal costal costochondral to make thoracic wall upward and enlarge thoracic space for repositinning of ectopic heart and using artificial patch to repair defect of diaphragm when necessary.