Saddle nose deformity is most commonly caused by trauma or iatrogenic intervention. Among other causes, that may be the reason of referring the patient to otolaryngologist, granulomatosis with polyangiitis (GPA) stands out. In upper respiratory tract manifests itself by rhinosinusitis with bloody-purulent discharge, crusting, nasal cartilage destruction and saddle nose deformity. Furthermore, chronic otitis media and interna can be observed that appear as otalgia, worsening or loss of hearing, tinnitus and vertigo. We report a case of 73-years old woman with suspicion of GPA, pointing out the difficulties accompanying the diagnostic process.