Congenital heart disorders (CHDs) are an important health issue due to heavy costs and emotional effects they impose to families and society. In general, the prevalence of CHDs is approximately 8 in 1000 newborn, with a multifactorial origin. On the other hand, previous studies have shown that the prevalence of CHDs is high among the children of parents with consanguineous marriage. The aim of this investigation was to determine the frequency of CHDs among the children of parents with consanguineous marriage in comparison with non-consanguineous parents. 605 medical records of children with CHD admitted at Imam Reza’s hospital, Mashhad, Iran during the years 2001 to 2005 were examined and questionnaires were completed and data were analyzed using a statistical software. The mean age of affected children was 1.25± 4 years. The average age of left obstructive acyanotic group was significantly higher than others (P< 0.001). Acyanotic disease with left to right shunt (%49) and cyanotic with decreased pulmonary flow (%19) were the most common defects. Other most common lesions were ventricular septum defects (VSD), atrial septum defects (ASD) and patent ductus arteriosus (PDA). Our data revealed that the risk of CHD increases with parental inbreeding, but there was no significant relationship between parents inbreeding and the type of CHD.