Medullary carcinoma of the thyroid is an uncommon tumour. In most patients, it is confined to the neck with or without involvement of the local cervical nodes. It rarely metastasizes to the mediastinum, lungs or liver. Intracranial metastasis is extremely rare with very few reported cases in the literature. We report an unusual case of an intracranial metastasis from a medullary carcinoma of the thyroid that occurred 25 years after primary surgery. We discuss the unusual features of our case together with a review of the literature.