Eighty-three patients with diagnosis of immunodeficiency were studied, antibody deficiency disorders were the most common 74.6%, abnormalities of unspecific mechanisms in 13.3% and deficiencies of cell mediated immunity in 9.6% of patients. Selective IgA deficiency was the most common of the humoral defects, 28 patients, the others were IgM and IgG selectives deficiency. The mortality was 6%, specially in patients with cellular deficiency. Immunodeficiencies are one of most common genetic diseases, needing appropriate diagnosis, treatment and developing centers with advanced technology.