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Abstract: Objective　To explore the clinical characteristics，causes of diagnostic delay and treatment response of nontuberculous mycobacterial（NTM） pulmonary disease in children with immunodeficiency，and provide reference for early clinical identification，diagnosis and treatment. Methods　Clinical data of 4 children with immunodeficiency complicated with NTM pulmonary disease admitted to the Department of Respiratory Medicine of Beijing Children’s Hospital from January 2019 to 
December 2025 were collected and retrospectively analyzed. Results　Among the 4 children，3 were male and 1 female，with onset age of 4-14 years. Underlying diseases included GATA2 deficiency，hyper-IgM syndrome after hematopoietic stem cell 
transplantation（HSCT），chronic granulomatous disease（CGD） and autoimmune lymphoproliferative syndrome（ALPS）. NTM strains were Mycobacterium kansasii，Mycobacterium abscessus and Mycobacterium avium-intracellulare complex. All children were misdiagnosed for 1-9 months before confirmation. All improved after anti-NTM treatment；during 2-60 months of followup，1 case had no recurrence after 24 months of standard treatment and drug withdrawal，1 case was lost to follow-up after 12 months of treatment，and the rest two cases remained stable under follow-up. Conclusion　The children with immunodeficiency are at high risk of NTM lung disease，which can be an important diagnostic clue for potential immunodeficiency. Metagenomic next-generation sequencing（mNGS） can increase the early diagnostic rate，and individualized anti-infective therapy combined with reasonable immunomodulatory therapy can improve prognosis.

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