Down syndrome (DS) is a common chromosomal anomaly. Congenital heart disease (CHD) is main prognostic factor contributing to a favourable or unfavourable outcome in these patients. To study the patterns of congenital heart diseases in children with Down syndrome. Hospital based descriptive type of observational study of 70 patients conducted at SPMCHI, Jaipur from April 2012 to Sept 2013. There were 70 DS children; 46 (65.71%) male and 24 (34.29%) female. Congenital heart disease was detected in 47 (67.14%) DS children. Out of those, 95.74% were detected as acyanotic heart disease and 4.26% were cyanotic heart disease. Total 73 congenital heart defects were detected in 47 DS children with CHD. In these children, 46.81% have single defect, 31.91 % have 2 defects and 21.28 % have multiple defects. In total congenital heart defects ASD (41.10%) was the commonest congenital heart defect followed by PDA (21.92%) and VSD (15.07%). It is imperative to know about presence of congenital heart disease in Down syndrome children. Contrary to result of most of other workers who observed atrioventricular septal defect was commonest defect. In present study ASD, PDA and VSD were the commonest CHD in order of frequency