It is usually associated with myeloperoxidase (MPO) ANCA or proteinase-3 ANCA.[1] AAV patients typically present with constitutional symptoms besides specific organ symptoms, such as fatigue, weight loss, and fever.[1] Various organs and systems can be involved, including ear/nose/throat, trachea, or lungs, kidney, skin, and nervous system.[2] Central nervous system (CNS) involvement occurs in less than 15% of AAV patients, presenting as hypertrophic pachymeningitis, cerebrovascular events, hypophysitis or mass lesions.[2–5]. This evidence concerns the gene MPO and hypophysitis.