Patient:71-year-old Caucasian woman, BCVA 0.3 at OD, 0.3 at OS; IOP 12/14 mmHg. Ocular Medical History: 2-year history of redness, irritation, and foreign body sensation in both eyes, treated with topical corticosteroids without improvement. General medical history: presented due to ocular symptoms but, on initial evaluation were found systemic manifestations consistent with Wegener granulomatosis, diagnosed on the basis of clinical and histopathologic features. The patient was treated with Rituximap 1000 mg and oral prednisone, 25 mg/day. Recurrent pulmonary inflammation developed and serologies for cytoplasmic-pattern antineutrophil cytoplasmic antibodies (C-ANCA) became positive. Main complaints: ocular irritation for the previous year, conjunctival and scleral ulceration of the left eye PURPOSE: To describe a patient with Wegener granulomatosis, presented due to ocular symptoms but, on initial evaluation were found to have occult systemic manifestations. Findings: Colour photography anterior segment (OS): diffuse conjunctival injection, focal vasculitis manifested by scleritis. No peripheral corneal thinning, anterior chamber quiet and deep. Discussion: In Wegener granulomatosis focal vasculitis is manifested by conjunctivitis, episcleritis, scleritis, corneoscleral ulceration, uveitis, and granulomatous vasculitis of the retina and optic nerve. Although ocular manifestations are relatively common in Wegener granulomatosis, the eyelid and its conjunctival lining are rarely involved (1). When this area is the initial focus of the disease, diagnosis may be difficult. Wegener granulomatosis has a variety of uncommon initial presentations. Although conjunctival involvement in this condition is rare, the diagnosis of Wegener granulomatosis should be suspected when the conjunctival inflammation is recurrent and not typical of other conjunctival inflammatory conditions. Wegener's granulomatosis (WG) is an etiologically obscure entity with multiple systemic manifestations. Ocular involvement is present in up to 58% of patients with WG. Harper et al (2) reported from 47 patients diagnosed with WG: 57.4% had systemic disease first and subsequently developed an ocular lesion, 6.3% had ocular inflammation first and then subsequently developed systemic manifestations of WG, 6.3% presented due to ocular symptoms but, on initial evaluation were found to have occult systemic manifestations consistent with WG, 30% had ocular lesions and no history or presence of systemic disease. He concluded, that ocular inflammation can occur with or without obvious systemic manifestations of WG. Ocular inflammation may represent the first sign of WG that enables the knowledgeable physician to diagnose this potentially lethal disease. Literature: (1) Jordan DR, Addison DJ. Wegener's granulomatosis. Eyelid and conjunctival manifestations as the presenting feature in two individuals. Ophthalmology. 1994 Mar;101(3):602-7. (2) Harper SL, Letko E, Samson CM, Zafirakis P, Sangwan V, Nguyen Q, Uy H, Baltatzis S, Foster CS. Wegener's granulomatosis: the relationship between ocular and systemic disease. J Rheumatol. 2001 May;28(5):1025-32.