Introduction:

Primary immunodeficiencies are genetic disorders of the immune system that increase susceptibility to infections. One of the most serious forms in children is severe combined immunodeficiency.

Case presentation:

This is the report of the case of an 8-monthold male patient who was diagnosed with severe combined immunodeficiency. The child presented a clinical profile consisting of multiple hospitalizations due to cytomegalovirus infection, endocarditis by Candida albicans and recurrent urinary tract infection by Pseudomonas aeruginosa.

The immune profile showed a decrease in the absolute number of CD3+ and CD19+ cells, which led to the diagnosis of severe combined immunodeficiency. Even though management was established, the child did not recover and died.

Conclusions:

The primary immunodeficiencies are disorders that require timely intervention to provide a better prognosis to patients.

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