A case of a massive cholesteatoma of the frontal sinus in an 80-year-old female is reported. This lesion is very uncommon and a review of the English literature revealed only 12 reported cases. Characteristically, these tumors present unilaterally as a painless, slow-growing mass, often accompanied by frontal headache, proptosis and diplopia, and without a history of frontal sinus infection. Curative treatment includes total excision of the cholesteatoma with adequate drainage, which leaves the patient with an excellent prognosis although late recurrence has been reported. While this type of lesion is very rare, it is nevertheless an important differential diagnosis of frontal sinus tumors, due to the associated destruction of bone.