A prospective clinical study was designed to establish the risk factors, the prevalence, and the progress of congenital heart defects in children with neural tube defects. Study included 90 children with a mean age of 13.5 +/- 30.4 months. There were 53 (59%) patients with spina bifida occulta and 37 (41%) patients with spina bifida aperta. The overall prevalence of congenital heart disease was 27.8% (40.5% in spina bifida aperta and 18.9% in spina bifida occulta; P = .024). There was no statistically significant difference for maternal age, usage of periconceptional folate, and maternal diabetes between the patient and control groups. The authors conclude that congenital heart defects are more common than reported in neural tube defects, and screening echocardiograms are warranted. This should be kept in mind especially in patients requiring minor or major surgical procedures. Furthermore, routine obstetric examination and therefore the use of periconceptional folic acid during pregnancy is still lacking in our country.