Four neonates with scimitar syndrome are presented. Three of the cases had congenital cardiovascular abnormalities not usually regarded as part of the scimitar syndrome, namely, ventricular septal defect, abnormalities of the aortic arch, and abnormal relationship of the pulmonary arteries and bronchi. Review of the literature indicates that among subjects of all ages the incidence of additional congenital heart disease is 24%; in patients within the pediatric age group the incidence is about 36% and is highest (75%) among those subjects having diagnostic studies while neonates.