Wegener’s granulomatosis (WG) is an uncommon condition characterized by necrotizing granulomatosis of the upper and lower respiratory tract and glomerulonephritis. Clinical symptoms and organ involvement of the disease vary widely. In this report we describe two cases of WG with unusual clinical presentations: one presented with erosive monoarthritis of the right knee and the other with serous otitis media. These symptoms were preceded by renal and pulmonary involvement. We think that this report will increase the awareness about the unusual clinical presentations of WG. This report also highlights the importance of early aggressive management to avoid further severe complications in WG.