Chronic granulomatous disease (CGD) is an inborn error of immunity (IEI). NADPH oxidase is an enzyme complex that produces various reactive oxygen species, such as superoxide anions and hydrogen peroxide. Pathogenic variants in cytochrome B (CYB)-B, CYBA, CYBC1, neutrophil cytosolic factor (NCF)-1, NCF2, and NCF4 genes of the NADPH oxidase enzyme complex are responsible for the clinical phenotype of CGD [1]. CGD has an X-linked or autosomal recessive inheritance pattern [1]. Patients with CGD present with various inflammatory and infectious diseases [1]. They are susceptible to fungal and bacterial infections, including mycobacterial infections [1]. Mycobacterium tuberculosis infections have been observed in Bacillus Calmette–Guérin (BCG)-vaccinated patients with CGD [2]. Mycobacterial infections in CGD are commonly observed in countries with a high prevalence of these microorganisms, such as those receiving the BCG vaccination at birth or those with a high prevalence of tuberculosis [2]. Nontuberculous mycobacterial (NTM) infections are rare in CGD [2]. Here, we described the first M. chimaera infection in a female patient with autosomal recessive CGD caused by a pathogenic variant in CYBA.