Background: Surgical management of patients with Atrioventricular Septal Defect (AVSD) has advanced over the last decades. The aims of this study were to evaluate the early and late outcomes in patients undergoing operation at a single heart center. Methods: Between April 2006 and April 2016, 337 consecutive patients underwent Atrioventricular Septal Defect (AVSD) repair at the Shaheed Rajaie Heart Center. The patients were subtyped as 73 (21.7%) partial, 67 (19.9%) transitional and 197 (58.5%) complete. Results: At the time of preoperative evaluation, there were 39 cases (11.7%) with moderate to severe or severe Left Atrioventricular Valve Regurgitation (LAVVR). Previous Pulmonary Artery banding (PA banding) had been performed in 144 (42.7%) patients and associated malformations were found in 115 (34.1%). Type of surgical repair was single patch 193 (57.3%), double patch 144 (42.7%), and single atrial septal defect patch with primary ventricular septal defect closure in TAVSD 64 (95.5%). 141 patients (71.6%) with CAVSD underwent definitive early repair by using a two patch technique and complete cleft closure. Annuloplasty and/or commissuroplasty were performed for 60 (17.8%) patients and debanding was also implemented in all patients. Mean hours for ventilation were determined as 26.8 ± 2.8. Mean days for intensive care unit was defined to be 4.4 ± 0.2, and total hospitalization time was as 26.8 ± 0.5. A 1 month, 21.9% (72 of 328) had ejection fraction less than 55%; 33 (10.9%) had more than moderate LAVVR; 73 (24.2%) had residual Ventricular Septal Defect (VSD), followed by 14 cases (4.6%) with residual Atrial Septal Defect (ASD) and 2 cases (0.6%) with Left Ventricular Outlet Tract Obstruction (LVOTO). Conclusions: Definitive early repair for AVSD can be performed with acceptable results. Despite a complete cleft closure, LAVVR remains the most common residual defect that more frequently required reoperation in the future.