Objective: To report a case of an X-CGD diagnosed patient presenting atypical zygomycosis. The X-CGD is a genetic disorder, autosomal recessive linked to the X, characterized by recurrent infections and granulomas formation. Description: Male patient, five years, admitted at the hospital experiencing fever and shortness of breath for two months. The patient was hospitalized and given Ceftriaxone and discharged after improved clinical condition, butcontinued showing dyspnea and fever. The persistent clinical state demanded lung biopsy which revealed mucorales fungi. Comment: This study has shown the importance of the correlation among infectious diseases from distinct pathogens in one individual.