A case of oncocytoma of the larynx is presented. These lesions are rarely found in the larynx, and have been more commonly described in the salivary and thyroid glands. The histopathological diagnosis is often difficult and the light microscopy can be unreliable for oncocytic lesions. For a more accurate definition of these tumours, the use of histochemical stains together with electron microscopy are advocated. With adequate surgery, the prognosis is excellent, and tumours are unlikely to recur. This paper discusses the management of a patient with a benign oncocytoma of the true vocal cord, and presents a review of the world literature.