Summary. This study characterized the clinical records of patients diagnosed with Congenital Upper Extremity Anomalies (CUEA) in a reference center in the care of congenital malformations in the city of Ibague, Tolima, Colombia. Methods: A descriptive, retrospective study was done. We included all clinical records of patients diagnosed with CUEA. The CUEA were classified according Swanson modified by the International Federation of Societies for Surgery of the Hand system (Swanson/IFSSH). Means and percentages were calculated from the CUEA, gender and site distribution, frequency of associated deformities, laterality, surgical intervention, number of interventions and comorbidities. Results: A total of 197 cases (55.7% male and 44.3% female). The average age of consultation was 7.82 years ± 6.69; 40.7% came from Ibague and the rest from the neighboring municipalities and other departments. The most common CUEA was radial polydactyly (16.02%), followed by central ray deficiency (14.72%), and cutaneous syndactyly (11.26%). We estimated crude incidence rate for the department of Tolima in 2001 of 7.8 per 10,000 live births, in 2002 of 8.6 per 10,000 live births and in 2004 6.5 per 10,000 live births. Conclusions: Given the implications in the functionality, cognitive development and labor productivity of patients with CUEA, it is recommended to implement a surveillance system that allows discrimination of the congenital deformities, according to their anatomical location, so as to facilitate analysis and to define appropriate behaviors.