PRTN3 and granulomatosis with polyangiitis: This case highlights the rare co-occurrence of cranial and spinal dural involvement in AAV, providing new clinical evidence to expand the understanding of AAV-related dural manifestations and improve diagnostic awareness among clinicians.<h4>Patient concerns</h4>A 57-year-old male presented with fever, headache, otalgia, and hearing loss, and was diagnosed with granulomatosis with polyangiitis based on positive anti-proteinase 3 antibodies (664.3), cytoplasmic anti-neutrophil cytoplasmic antibody (1:10).