Introduction: Primary immunodeficiency is a group of genetic disorders manifested as an improper activity of immune system elements. Its most common clinical signs are recurrent infections. Children are treated with immunoglobulin replacement therapy, which they can receive intravenously or subcutaneously. Parents also play an important role in the child’s care therefore a nurse must include them into the treatment plan. Aim of this thesis: To introduce primary immunodeficiency diseases in child. The objective of this study is to describe primary immunodeficiency, treatment with immunoglobulin, a nurse’s role in the treatment and the quality of life with such a disease. Methods: A descriptive method of work was used based on a review of foreign and domestic scientific and professional literature published between 2010 and 2016. Remote access databases CINAHL, Google Scholar, MedlinePlus were used. Discussion and conclusion: Primary immunodeficiency diseases have become a growingly recognizable group of diseases through years. Treatment options are improving and they enable a better quality of children's lives and their greater autonomy. A nurse has to prepare treatment and make sure it is appropriately applied. She also has an important role in the health education, giving support, helping the parents and children who have to face a new kind of responsibility due to their disease.