Conidiobolus lamprauges (C. lamprauges) is a rare pathogen that causes conidiobolomycosis, resulting in high mortality and difficulty in clinical treatment. There has been no report of pulmonary infection caused by C. lamprauges in China so far. This paper reports for the first time a case of anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis patient complicated with C. lamprauges pulmonary infection, aiming to enhance the control capabilities for this disease in clinic and laboratory diagnostics.