OBJECTIVE: Given the rare occurrence of this disease, the aim of this work is to describe a case of Wegener's granulomatosis accompanied by a literature review. CASE REPORT: A 48-year-old woman reported that 60 days prior, her symptoms began with otalgia and hearing loss in her left ear. Later, the symptoms evolved to include purulent rhinorrhea with nasal obstruction, increased hearing loss, and occasional episodes of epistaxis. In the otoscopy examination, serous otitis media was observed. After consulting with an otolaryngologist, she underwent a CT scan of the sinuses, which showed left pansinusitis. Drainage was performed, with the secretions appearing fungal, and bone erosion of the maxillary sinus wall was observed. A diagnosis of fungal rhinosinusitis or Wegener's granulomatosis was assumed, and treatment with amphotericin B was commenced. A biopsy of the left maxillary sinus was performed, and laboratory tests, lying granulomatous lesions, and a c-ANCA test yielded positive results. Chest radiography showed bilateral pulmonary infiltrates, progressing in 2 days with bilateral pleural effusion. The patient was treated with cyclophosphamide and prednisone, showing improvement. CONCLUSION: Wegener's granulomatosis is a necrotizing vasculitis of medium and small vessels, with the formation of granulomas. The disease is more common in whites, has no gender predominance, and the mean age of onset is between 30-50 years. When it presents as a classical triad with involvement of the upper airways, lungs, and kidneys, the sensitivity of c-ANCA reaches 97%. The specificity of the standard c-ANCA test for Wegener's granulomatosis is high, reaching 98%.