Introduction:Hereditary angioedema (HAE) is a primary immunodeficiency with a rare autosomal dominant pattern of inheritance, characterized by a quantitative and/or qualitative deficiency of C1-inhibitor (C1-INH) mainly due to genetic mutations. It is characterized by presenting edemas in the skin and in the mucosa of different organs, mainly the digestive tract and the respiratory system. The clinical manifestations can be mild or severe, depending on their intensity and location.Objective: To know the epidemiological frequency and clinical and laboratory datas on patients with hereditary angioedema (HAE) in Cuba. Methods:The criteria for the phenotypic diagnoses of PID in this project were taken of Latin American Society Immunodeficiency Diseases (LASID) and it includes all diagnoses used by the World Health Organization (WHO) and the European Registry for Immunodeficiency Diseases (ESID) and the most recent classification developed by the International Union of Immunological Societies (IUIS) PID Classification Committee. Pediatric and adult-made clinical histories were used for project purposes that record the variables: demographic information, clinical and phenotypic characteristics, molecular defect, inheritance, associated diseases, nutritional status, quality of life, form of presentation, types of infections and laboratory tests used for diagnosis and treatment.Results:42 patients were diagnosed in 9 provinces of the country. 34 patients were classified as type I and 8 with type II. Abdominal disorders were present in 31 patients, one patient with seizures and 25 with edema of the glottis. 19 patients were receiving long-term prophylactic medication, of whom 15 received danazol and 8 tranexamic acid. Eight patients were treated with Berinert, especially pregnant women at term and in the prepartum period, and another 14 patients in crisis with fresh plasma due to poor drug availability Conclusions:To know the diagnosis and clinical characteristics of each patient will improve the management of the disease and will expand the therapeutic possibilities of these patients in Cuba.