Wegener’s granulomatosis (WG) is characterized by necrotizing granulomatous vasculitis affecting mainly small vessels of the upper and lower respiratory tract and kidneys. The aim of this article is to present diagnostic and therapeutic problems connected with WG especially with negative c-ANCA antibodies. A case of a 34-year-old woman is described. Initially she was complaining of hearing problems and was presenting with otitis media symptoms. Diagnostic tests revealed proteinuria and diffuse lung involvement with numerous nodules. WG was diagnosed only 9 months after first symptoms appeared based on the histopathological examination of the lung sample obtained from the videothoracoscopic biopsy. The appropriate treatment was started and a fast health improvement was achieved.