a b s t r a c tObjectives: We aimed in our study to determine the incidence and type of congenital cardiac anomaly (CCA) in newborns associated with imperforate anus (IA), the outcome of surgical reconstruction of the anorectum in our center, and the need for performing echocardiography in all patients with IA. Materials and methods: The preoperative echocardiography reports of all cases born with IA and managed at King Abdulaziz University Hospital, Jeddah, Saudi Arabia over a period of 11 years (Jan 2000eDec 2010) were reviewed. The average annual delivery rate of this hospital is 5500. Results: During the study period, 61 patients of IA were diagnosed that showed an incidence of about 1 per 992 live births, and the rate of CCA among the IA subjects was 15 (24.6%). In 12 patients (19.6%), the associated CCAs were of a mild nature, and reconstruction of the anorectum went smoothly. Three patients (4.9%) had significant CCA and died. Conclusion: The incidence of IA in our hospital is 1 per 992 live births, and its association with CCA is 24.6%. The majority of CCAs associated with IA were of the mild type.