BACKGROUND: In developing countries, infants with Down syndrome and cardiac defect  are at increased risk of dying. Congenital heart diseases occur in 40-50% of affected infants. Endocardial cushion defect accounts for the most. Pattern of cardiac defects in Down syndrome vary  with ethnicity. The current study aims to determine pattern of  cardiac defects and survival of patients in our institution. METHODS: Hospital based review of cases, between April 2010 and may  2015 were made.  Data were analyzed  using SPSS version 20 software quantitatively with plotted Kaplan Meier survival curve done. RESULTS: Down syndrome cases, 53 male and 63 females with cardiac anomalies, were described. Patent ductus arteriosus occurs in 57(36.5%), Ventricular septal defect in 31(19.9%), Atrial septal defect in 30(19%), Atrio-ventricular septal defect in 29(18.6%),Tetralogy of Fallot in 4(2.6%) and others in 5(3.2%) cases. Cases were  alive, lost to follow-up and died in 59, 35 and 22 cases, respectively. The overall death rate was 19% and two-third of them died during infancy with females dying at an earlier age than males. Pulmonary hypertension, hypothyroidism and gastrointestinal disorders  were diagnosed in 46, 21 and 4 of the cases, respectively. Reasons for lost to follow-up were discussed. CONCLUSION: The pattern of cardiac anomalies in our study among Down syndrome cases is dominated by Patent ductus arteriosus while, the overall survival of cases is guarded as majority of deaths occurred during infancyKEYWORDS: Down syndrome, cardiovascular anomaly, congenital heart disease, trisomy 21.