Midline granuloma is a very rare syndrome, associated with a constant and progressive ulceration of the centrofacial tissues and paranasal sinuses. The diagnosis is difficult due to inespecific clinical aspects and sometimes is necessary several biopsies to confirm the exact diagnosis. Analyse 17 patients diagnosticated and treated in the HC FMUSP in the last twenty years. The charts of 17 patients with syndromic diagnosis of midline granuloma were reviewed and we analysed the hystologic type, sex, age, clinical aspects, treatment and follow up of these patients. We found 3 patients with Wegener\'s granulomatosis, 4 with polymorphic reticulosis, 3 with non Hogdkin lymphoma and 7 with idiopathic midline granuloma. The mean age was 39 years and 82% were male. The principal clinical aspects were nasal obstruction, local infection, facial deformity and bleeding. Treatment includes surgery and/or radio or chemotherapy depending on the hystologic type. During follow up, 3 patients died, 4 had recurrence of the disease, 5 did not recurrence, and in 5 patients we lost the follow up. Midline granuloma is an extremaly agressive disease, with difficult diagnosis and bad prognosis. The initial clinical suspect is important to start a specific treatment improving the survival of these patients.