The myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) test was positive, and the young patient gradually developed palisaded neutrophilic and granulomatous dermatitis (PNGD), orbital and paranasal sinus granulomatous neoplasms, along with intermittent nose, head, and orbital pain. Here, MPO is linked to granulomatous dermatitis.