A review of 78 cases of neurofibromatosis in children disclosed 6 with congenital heart disease. This included 2 cases of pulmonic valvular stenosis and 1 case each of coarctation of the thoracic aorta, atrial septal defect, ventricular septal defect and congenital heart block. This material, therefore, further emphasizes the increased incidence of congenital heart disease in patients with neurofibromatosis.