Background and Aims Preventive strategies of congenital anomalies are basically relying on the systematic ongoing collection and analysis of data and timely dissemination of information. The aim of this paper is to briefly report a critical review of a surveillance system of congenital anomalies in a developing country, by describing the challenges and experience of the registry since it began. Methods Tabriz Registry of Congenital Anomalies (TRoCA) was mainly set up based on the guidelines provided by the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR) for data collection, coding, process, analysis, use, and evaluation of the system. Findings TRoCA has successfully achieved its main objective as a pilot model for setting up a nationwide registry of congenital anomalies in the country. The programme has too succeeded in relation to its regional objectives: epidemiological rates and data have been produced consistently for etiological investigations, methodological studies, service provision, and preventive measures for selected anomalies. Conclusions Our successful experience, as a small registry in a developing country, might be of interest and useful to practitioners, policymakers of birth defects control programmes, and mainly those willing to set up a monitoring system of congenital anomalies in similar areas.