Purpose - to analyze the currently described variants of the course of COVID-19 in immunocompromised patients in order to inform the medical community and focus on this problem. Two clinical cases of different course of COVID-19 in patients with primary immunodeficiencies, both mild, asymptomatic and severe, with a fatal outcome, are presented. In the first case, in a child with Nijmegen breakage syndrome, despite lymphopenia, the course of SARS-CoV-2 was asymptomatic, which may be due to the regular administration of immunoglobulin for replacement purposes. The peculiarity of the second case was a repeated episode of COVID-19 in an immunocompromised child with APECED. The first episode of COVID-19 in September 2020 had a mild course, but led to the manifestation of immunodeficiency symptoms. The present symptoms (retinopathy, hepatitis), except for mild mucosal candidiasis, are not part of the triad of classic APECED symptoms, although along with the reaction to the live vaccine, they made it possible to suspect immunodeficiency. Immunosuppressive therapy contributed to stabilization of hepatitis, but ocular symptoms were without positive dynamics. The second episode of COVID-19 was observed in January 2022. It proceeded with prolonged fever for 2 weeks, which was resistant to treatment, with progressive cytopenia, hypoproteinemia, hypoalbuminemia, signs of active hepatitis, hyperferritinemia, elevated triglycerides; coagulopathy with low fibrinogen levels. Subsequently, signs of pneumonia were added, confirmed by radiology. Another feature of this case was the presence of a mixed infection - COVID-19 and Epstein-Barr virus infection. The cause of death in this case was not only COVID-19 pneumonia, but also progressive macrophage activation syndrome. Conclusions. Thus, the sequence of COVID-19 in patients with inborn errors of immunity can be either asymptomatic and mild or fatal, depending on the type of immune disorders, the therapy received, and concomitant conditions. SARS-CoV-2 in immunocompromised individuals requires close attention for timely diagnosis of life-threatening conditions. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.