Abstract: To describe the diagnosis, management and outcome of children with heart disease seen at Sudan Heart Centre (SHC). This study reviewed all Children with Heart Diseases (CHD) seen at SHC by one paediatric cardiologist from July 2004 to June 2005. Patients were evaluated clinically and by echocardiography. Cardiac catheterization was done when indicated. Patients were operated on by one paediatric cardiac surgeon from SHC and by a visiting paediatric cardiac surgeon. Five hundred and twenty two patients were evaluated, 435 had abnormal hearts. The median age was 48 months (one day-15 years). Congenital Heart Disease (CHD) constituted 87% and acquired heart disease and rhythm disorders 13%. The frequency of the different CHD was described and in general did not differ from that reported in the literature except for Ebstein anomaly which was noted to be 4 times more frequent than reported. Cardiac catheterization was done for 81 patients, for diagnosis in 61 (75%) and for intervention in 20 (25%) patients. Interventions included balloon dilatation, atrial septostomy septal defect and patent ductus arteriosus closure with the help of a visiting team, the success rate for interventions was 95%. Surgery was done for 125 patients. Seventy three operations were done by the local team and 52 by the visiting team. The 30 day operative mortality was 8.3%. Paediatric cardiac service in Sudan in echocardiography and cardiac catheterization is growing. Cardiac surgical results are comparable to the study.