Granulomatosis with polyangiitis (GPA) is a subtype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) that commonly requires aggressive immunosuppression to achieve remission. We present a case of a young Malay lady with recurrent episodes of ANCA-positive nodular anterior scleritis who responded poorly to topical and systemic corticosteroids and relapsed while on methotrexate. A year later, she had epistaxis, and a sino-nasal biopsy confirmed granulomatous vasculitis. While receiving cyclophosphamide, she developed proptosis with optic neuropathy, which resolved with intravenous methylprednisolone. She eventually required rituximab as she was still having relapses on other immunosuppressants. Adequate and targeted treatment with immunomodulators is crucial to achieving disease remission in GPA.