Case report peripheral ulcerative keratitis with nodular scleritis, presents as the primary manifestation of granulomatosis with polyangiitis.

Scleritis and peripheral ulcerative keratitis can be the manifestation of a GPA. Careful evaluation, diagnosis, and management with a multidisciplinary medical team, early start of cyclophosphamide, and rituximab have a huge role in decreasing the disease activity and is life-saving.