We analysed the incidence and spectrum of congenital heart disease (CHD) in the Sultanate of Oman from 1994 to 1996. CHD was detected in 992 of 139,707 live births (incidence 7.1/1000 live births). The common CHDs were ventricular septal defect (24.9%), atrial septal defect (14.4%) and patent ductus arteriosus (10.3%). The frequency of atrioventricular septal defects (5.9%) was higher than reported from other countries. Age at diagnosis was under 1 month in 38% and 1-12 months in 40%. Cyanotic CHD was found in 21.7% of the whole group and 35% of neonates. Although this was a hospital-based study, we believe we included almost all the infants and children with CHD in the country. The incidence and pattern of CHD in Oman were similar to those reported from developed countries in Europe and America, except for a higher frequency of atrioventricular septal defects. The high prevalence of consanguinity in the country did not affect the overall incidence of CHD.