OBJECTIVE: To assess age, gender distribution and relative frequency of congenital heart disease (CHD) in children who underwent palliative or corrective cardiac surgery at Rehman Medical Institute, Peshawar. METHODOLOGY: This retrospective study was conducted in Department of Cardiac Surgery at Rehman Medical Institute (RMI), Peshawar from May 2008 till May 2010. One hundred and twenty three patients up to age group of 16 years with confirmed diagnosis of congenital heart disease admitted for Cardiac Surgery at Rehman Medical Institute, Peshawar were included. RESULTS: Out of 123 patients, there were 71 males (57.7%) and 52 females (42.2%), with male to female ratio of 1.3:1. Sixty five (52.8%) of the total cases had acyanotic heart defects. Cyanotic heart defects were seen in 58 patients (47.1%). Ventricular Septal Defect (VSD) followed by Patent Ductus Arteriosus (PDA) and Atrial Septal Defect (ASD), were the commonest acyanotic heart lesions, 33.8%, 23.0% and 16.9% respectively. Tetralogy of Fallot (TOF) was the commonest cyanotic lesion. Conclusion : Majority of patients with congenital heart disease had acyanotic CHD with the commonest lesion being VSD. Tetralogy Of Fallot (TOF) was the commonest cyanotic lesion. Most of the patients were less than five years with no significant difference in sex distribution. Availability of expertise locally will lead to more patients getting surgical treatment at an earlier age thereby reducing morbidity and mortality and improving quality of life for these children.