The article provides a description of clinical case of patient 53 years old with Wegener's granulomatosis, manifested with necrotizing scleritis of the left eyeball against the background of malignant nose granuloma. The patient underwent sclera plastic surgery twice in order to save the eye. Due to intensive corticosteroid and cytostatic therapy clinical activity of vasculitis was reduced. At the same time was observed further growth of nose granoluma with damage of inferior orbital wall and spread of the tumor to the orbital cavity with the subsequent destruction of the eye. It was performed an enucleation of the left eye with removal of the tumor of the left orbit. The Wegener's granulomatosis diagnosis was confirmed histologically. The problem of treating systemic vasculitis and its complications is very relevant. During 5-year observation period the patient there were noted another complications: septal perforation, oroantral fistula formation, formation of bilateral hearing loss, disintegration of the left lung granuloma. Right eye stayed intact during whole observation period. Prognosis is unfavorable not only for eyesight, but also for patient's life. Keywords: Wegener's disease, granulomatosis with polyangiitis, necrotizing sleritis