Abstract
Pachymeningitis is a rare manifestation of granulomatosis with polyangiitis (GPA), and there is a paucity of literature regarding the efficiency of rituximab (RTX) in such cases. We present a woman in her early 50s with GPA who initially responded adequately to conventional therapy. However, the treatment course was complicated by pulmonary cryptococcosis. The patient later developed non-infectious pachymeningitis, which failed to respond to steroids and methotrexate. However, using RTX upfront led to good clinical response and radiological resolution.