Congenital heart disease is an important cause of infant mortality and disability. The frequency, spectrum and contributory risk factors for significant cardiovascular malformations among live-births was retrospectively evaluated at the Aga Khan University Hospital. Of a total of 8331 live births between July, 1987 and December, 1992 34 babies were diagnosed to have congenital heart disease in the neonatal period giving a prevalence of 4 per 1000 live births. Ventricular septal defects was the most common (n = 10, 29%) abnormality. Eight cases had associated chromosomal abnormality, the most common being Trisomy 21. Maternal abortions, still-births, consanguinity and diabetes mellitus were not found to be significant risk factors for congenital heart disease in this survey