Angiocentric immunoproliferative lesion(AIL) in the upper airway is notorious for its dismal clinical course. To better assess the prognosis of this uncommon disease, thirty-four patients who were diagnosed by histopathological and immunohisochemical methods during the period beginning in March 1981 until February 1991 and who were followed for over 5 years were analysed. Retrospective chart review and analysis of treatment results were done with respect to the site of origin, clinical symptoms such as the presence of B-symptoms, extent of disease, and various treatment modalities. Mean 5 year survival was found to be 38%. The nasal cavity was the most frequently involved site and Bsymptoms together with extensively involved tumor contributed to worse prognosis. A statistically significant difference in prognosis was seen between different therapeutic modalities and better outcome was seen in the combined chemotherapy and radiation group. In case of treatment failure, mean life expectancy was 26±8.45 months. Common causes of death were distant and/or systemic metastasis, sepsis, repiratory distress and renal failure. Because AIL is often presents as a localized disease, it is managed by radiation. However, to improve survival by reducing distant failure rates, we believe that early combination of systemic chemotherpy may be useful.