BACKGROUND፡ Birth defects are conditions that exist at birth and cause structural changes in one or more parts of the body. In order to plan proper management and design preventive activities of these conditions, accurate tracking, registration and analyses of the registered data are important. We assessed the practice of birth defect registration at Addis Ababa health facilities.METHODS: We retrospectively checked the existence of a separate birth defect registry book and assessed the delivery room registration book for completeness in registering birth defects. We also assessed the total number of birth defects registered during 2010-2015. RESULTS: We assessed the practice of birth defect registration at 37 delivery service providing health facilities in Addis Ababa, 20 public and 17 private institutions.  Of the 37 health institutions assessed, 23 registered birth defects (3 of them used a separate birth defect registry books, and 20 used a regular registration book to register birth defects). The remaining 14 did not register any congenital anomaly. Of the institutions that do not register congenital anomalies, 10 are private and four are public.CONCLUSION: Only three delivery providing health facilities had a dedicated birth defect registry book which is close to ideal for a birth defect registration. There is a need for others to do the same until an electronic birth defect registration is established. This registration will serve as a resource for clinical governance and studies into quality of life, quality of care, etiology and prevention. KEYWORDS: Birth defect, Registration, surveillance