Objective To explore the clinical characteristics of systemic disseminated infection caused by Mycobacterium fortuitum (M. fortuitum), and improve the diagnostic rate and understanding of the disease. Methods One case of systemic disseminated M. fortuitum infection was reported, and analyzed in combination with relevant literatures. Results Patient was with multiple systemic involvement (including lung, lymph node, skin, joint), lymph node tissue culture was positive for M. fortuitum, patient was given clarithromycin+levofloxacin+linezolid for treatment, disease was remitted. Conclusion Systemic disseminated M. fortuitum infection is rare, and patient with GATA2 deletion and IFN-γ autoantibody may be a potential mechanism, diagnosis is mainly based on patholo-gical morphology and microbiological detection, but positive rate is low, diagnosis is difficult.