Aim To summarize the experience of one-stage surgical treatment of aortic coarctation (CoA) combined with ventricular septal defect (VSD) with extended end-to-side anastomosis in recent years. Methods The data from 208 cases with CoA combined with VSD admitted to hospital between January 2010 to October 2017 were collected and analyzed. There were 137 boys and 71 girls, the median age was 54 (5～730) days, the median weight was 4.0 (1.8～9.9) kg. Extended end-to-side anastomosis was used to correct the CoA, and the VSD was repaired simultaneously by an incision through median sternotomy. All operations were performed by the same group of surgeons. The data were analyzed by SPSS 20.0. Results Early death occurred in 6 cases (2.9%) and late death occurred in 3 cases (1.4%).Recoarctation developed in 16 cases (7.7%) and reoperation occurred in 19 cases (9.1%). The level of brain natriuretic peptide (BNP) increased remarkably before operation but showed no obvious change after operation. After operation, transcoarctation gradient pressure was reduced, and the transcutaneous oxygen saturation, C-reactive protein (CRP), lactate and creatinine were increased. Preoperative hypertension existed in 104 cases (53.0%), and after operation, blood pressure returned to the baseline level in 30 cases (28.8%) and continued to rise in 35 cases (33.7%). Higer transcoarctation gradient was the risk factor for postoperative recoarctation. Conclusions Correcting the CoA combined with VSD simultaneously with extended end-to-side anastomosis by an incision through median sternotomy was effective and safe, and the outcomes of immediate and medial-term were satisfactory, with low rates of mortality and recoarctation. Higer transcoarctation gradient pressure was the risk factor for postoperative recoarctation.