ObjectiveThe epidemiological profile of congenital anomalies of the upper limbs (CAULs) is of major relevance to monitoring and planning. A study of this profile may reveal if there is prevalence of some specific type of malformation in comparison to a more comprehensive epidemiological sample. The Latin American Collaborative Study of Congenital Malformations (ECLAMC) has an extensive database, providing an excellent source of comparison. This study aims to evaluate the epidemiological profile of CAULs at the hand surgery department of the Hospital Federal da Lagoa (HFL) in Brazil, and compare it to the ECLAMC data.MethodsWe conducted a retrospective analysis of patients who underwent treatment at the pediatric outpatient hand surgery clinic. The sample universe consisted of 126 patients (4 of these patients presented with 2 simultaneous anomalies), totaling 130 malformations.ResultsThe results demonstrated that the comparable pathologies have significantly similar incidence rates. It is worth noting the polydactylies (pre- and post-axial), where the percentile of incidence in the ECLAMC was higher.ConclusionThis study showed that the epidemiological profile of patients who underwent treatment at this hospital was equivalent to that found in the ECLAMC database. Level of evidence III, Retrospective epidemiological study.