BACKGROUND There have been rare reports of normolipidaemic xanthelasma in Wegener's granulomatosis. Herein, we describe another case associated with a review of the literature. METHODS A 62-year-old woman was hospitalized for Wegener's disease with cutaneous, ENT, renal, joint and neurological signs. Physical examination showed bilateral palpebral xanthelasma with infiltrated lacrimal glands that had appeared simultaneously to the other clinical signs. No hyperlipidaemia was noted. Biopsy of the xanthelasma revealed a typical infiltrate of foamy histiocytes and focal vasculitis. On treatment with corticoids and cyclophosphamide, the yellow lid lesions gradually disappeared. CONCLUSIONS Normolipidaemic xanthelasma is rarely reported in association with Wegener's granulomatosis. It accompanies local ophthalmological inflammation and its appearance during the course of this disease must be dealt with carefully.