Adenoid cystic carcinoma of the nasal cavity and paranasal sinuses has a unique natural history. It has a propensity for frequent local recurrence and early perineural and hematogenous spread. These factors, together with a complex anatomic situation, make adequate oncologic surgery extremely difficult. The incidence of local recurrence is lower in those patients receiving more radical primary surgery, but the choice between conservative and radical management may not alter prognosis. Indeed, it is doubtful if any patients are cured of this disease.