Background. Metastases from tonsilar cancers are uncommon, usually found in the lung and less commonly in the bone, liver, and mediastinal sites. Only approximately 20% of patients die from distant metastasis. Central nervous system (CNS) metastases usually appear later in the course of the disease, with only 1 % to 2% of patients developing metastasis involving the CNS in the course of their disease. Patients seen with symptomatic CNS lesions are rare. Methods and Results. A case report is presented of a patient seen with signs and symptoms of CNS disease from an isolated CNS metastasis. The primary site was ultimately determined to be of tonsilar origin. The patient's treatment and outcome are discussed. Conclusions. Tonsilar cancers seen with signs and symptoms of CNS disease is a rare event. The benefit of aggressive treatment of isolated CNS metastasis from head and neck cancers will likely be gained from case reports because the incidence is quite low. © 2008 Wiley Periodicals, Inc.