Congenital birth abnormalities are a major factor in infants' poor health, which affects their likelihood of surviving. Even though these visual anomalies are rare, they have a substantial influence on people's quality of life.This study aimed to describe the clinical profile of congenital ocular anomalies with different epidemiological parameters in a tertiary health center in western Orissa. The study examined 3674 patients in the age group from 0 to 14 years who were admitted to the V.S.S. Medical College, Burla, Odisha and included 151 eyes from 99 cases of various types of congenital anomalies. A detailed history and other necessarydata were collected by trained ophthalmologists and descriptive analyses were carried out. The incidence of congenital ocular anomalies was 2.69%. Nasolacrimal duct anomalies (47 eyes) were the most common congenital ocular anomaly (31.1%); followed by congenital cataract (41 cases; 27.3%) and colobomy eye (18 cases; 11.9%). There was a slight male preponderance that accounted for 56.57% of all cases.The majority ofpatients were younger than 5 years (68%) and theincidence of congenital ocular anomalies was highest in the 0-5 year age group (68.69%). Single ocular involvement was observed in 47 cases (47.47%) while bilateral involvement was observed in 52 cases (52.53%). Consanguinity was identified in 13.13% of cases, with an increased risk of bilateral involvement (84.6% of cases). The most common symptom was ametropia (38.4%), followed by tearing (32.3%) and a white pupillary reflex (14.1%). A total of 60 eyes (39.7%) required surgery. Congenital ocular anomalies are a common cause of ocular morbidity, accounting for 2.7% of all cases in the pediatric age group. Early diagnosis, referral, and appropriate intervention would help prevent most permanent sequelae in congenital ocular abnormalities