Aim: To determine the pattern of presentation of congenital ocular anomalies amongst children seen at the University of Benin Teaching Hospital. Materials and method: This study was a hospital based descriptive cross sectional study. A total of 372 respondents aged 15 years and below made up of 200 males and 172 females participated in the study. These patients were recruited on presentation to the Eye clinic of University of Benin Teaching Hospital, and also based on a referral from the General Practise Clinic, Children Emergency Ward, Children’s wards and the Special Care Baby Unit of the Hospital. All the patients who presented in the study period and met the inclusion criteria, were recruited for the study. Results: Congenital cataract (33.3%) was the commonest congenital ocular anomaly seen. Congenital esotropia (17.7%) and congenital glaucoma (13.6%) were the second and third commonest congenital anomaly seen. Other less common congenital anomalies seen were Blepharophimosis syndrome, congenital orbital cyst, congenital lid eversion and eyelid coloboma. A family history of congenital ocular anomalies and a history of fever in the first trimester of pregnancy were significant risk factors in having a child with congenital ocular anomaly. Knowledge on the cause of congenital ocular anomaly was generally poor, as 73.1% of the parents/caregivers did not know the cause. A p –value < 0.05 was taken as significant. Most of the children (65.3%) presented to the hospital before their fifth birthday. Conclusion/ recommendations: Congenital ocular anomalies are associated with a family history of congenital ocular anomalies and fever in pregnancy. The public should be educated i on the need for mothers to register for and attend antenatal clinic as early as possible in pregnancy. They also need to be educated on the importance avoid traditional and non prescription medications in pregnancy. They should also alert their physicians that they are pregnant when they are being prescribed medications in order to avoid medications that may be potentially harmful to the foetus.