Introduction: Congenital heart disease (CHD) is an abnormality in the structure or function of the cardiocirculatory system present at birth but more often diagnosed subsequently. CHD is the most common (28%) major congenital anomaly and thus signifies a major global health problem. The primary objective is to estimate the frequency and pattern of CHD in children in the Andaman and Nicobar Islands (India).Methods: We did a hospital-based retrospective observational study. The hospital case records of all children belonging to the age group of 0 to 12 years with newly diagnosed CHD were reviewed for the five years from January 1, 2016 to December 31, 2020. The clinical, demographic, and echocardiogram details were retrieved, and descriptive analysis was done using the Statistical Package for the Social Sciences (SPSS) for Windows Version 26 (IBM, Chicago, USA).Results: A total of 201 (12.8 per 1000) children were newly diagnosed with CHD (out of a total of 15592 children). There were 110 (54.7%) boys and 91 (45.3%) girls in the age group of 0 to 144 months (mean ± SD: 13.86±27.13). The ventricular septal defect (VSD) is the most common congenital heart defect, accounting for 25.4% of all CHD cases. The most common cyanotic CHD was tetralogy of Fallot (TOF), comprising 8% of the total cases. Conclusion:The spectrum of CHD in our study was largely similar to pre-existing literature. Although most of the CHDs were detected during infancy, a higher proportion of complex lesions in our study group resulted in adverse outcomes, even in surgically managed cases.