Successful reconstruction of an ocular defect resulting from granulomatosis with polyangiitis, following treatment with rituximab.

Purpose: To report a unique case of orbital inflammatory disease which was ultimately diagnosed as granulomatosis with polyangitis (GPA) and thus successfully treated.

Observation: A 47 year-old man presented with a rapidly progressive necrotic soft tissue mass within the medial antero-superior aspect of the right eyelid and orbit. He also had transient retinal vasculitis in the left.