Our work aims to the study of autoimmune changes in primary immunodeficiencies (PID) diagnosed during childhood. Patients: 54 children with different types of PID. Method: investigation of humoral immunity-immunoglobulins, complement C3, circulating antigen-antibody complexes; cellular immunity parameters CD3, CD19, CD20, CD4, CD8, CD3-DR, CD16 cells; study of autoimmunity markers direct and indirect Coombs test for erythrocytes, hemolysines, aglutinines, crioglobulins, lupus cells, anti-DNA antibodies, antiplatelets antibodies and other additional tests for the diagnosis. Results: autoimmunity was diagnosed in 4 of 22 cases with selective IgA deficiency (IgA-D) and in 4 of 19 cases with common variable immunodeficiency (CVID). In the group of IgA-D, 18 children had a symptomatic immunodeficiency syndrome, while 4 were asymptomatic. In the group of CVID, all children had more or less important clinical expression of the immunodeficiency syndrome. In the IgA-D the autoimmune complications were: autoimmune thrombocytopenia (3 cases) and rheumatoid poliarthritis (1 case). Three of these cases were asymptomatic from the point of view of congenital immunodeficiency. From the 19 cases with CVID, we noticed the following autoimmune diseases: haemolytic anemia (1 case), immunoneutropenia (1 case), autoimmune thrombocytopenic (2 cases). Conclusions: We noticed the high frequency and early onset of autoimmune diseases, especially in form of hematological diseases as complications of PID. We noticed the early occurrence at relative high frequency of autoimmune disorders in PID diagnosed in childhood most expressed as hematologic autoimmune disease.