This paper reports a retrospective record analysis of the last six years which was done in Damascus’ Maternity University Hospital, to determine the birth prevalence of birth defects, describe its pattern, and discuss methodological problems of such study. Of a total of 87638 births, 1158 cases of birth defects were found, giving a birth prevalence of 13.2/1000 livebirths and stillbirths (95% CI: 12.8-13.6). The most frequent defects were the neural tube defects which accounted for 51.6 % of total defects. A statistically significant linear trend of increasing NTDs was noticed. This study highlights the necessity of applying available strategies of preventing the preventable neural tube defects. It also suggests the establishment of special registry of birth defects to determine prevalence, trends and their determinants.