BACKGROUND: Congenital heart disease is an abnormality of anatomical structure of the heart that is of paramount importance. The incidence is approximately 8 per 1000 live births. CHD not only contributes to signicant mortality and morbidity but also causes tremendous psychological stress and economical burden to whole family. OBJECTIVE: To study the pattern and clinical prole of congenital heart diseases in a tertiary care hospital and to aid for early st detection and quick referral of children with congenital heart disease RESULTS: In this study, done between January 1 2020 to june 31st 2021, 54 children with conrmed congenital heart disease were observed. ACHD was the most common type of CHD and of them, VSD was the most common type, followed by ASD. Based on sex distribution, 31 were male (55.5%) and 23 were female (44.5%), with a male: female ratio of 1.25:1. Most children belonged in the age group 6m – 1year (46.2%) followed by (42.5%) in 30days – 6months age. The common clinical presentation observed in this study was breathlessness. The common complication observed in the current study was heart failure followed by malnourishment and recurrent LRTI.