Atrial septal defect is common congenital heart disease in children. Inheritance follows the multifactorial mode primarily but in certain families autosomal dominant transmission is evident. In Korea, there has been no case report of twins with ASD or any other congenital heart diseases in children, while there have been several reports of sildings and twins with ASD or other congenital heart diseases in other countries. Herewith we report a monozygotic pair of twins with atrial septal defects who were diagnosed by cardioc catheterization and confirmed by operation, with brief reriew of the literature.