Primary chordoma of the paranasal sinuses are extremely rare tumours, with only a small number of cases verified and published in the literature. It appears that only five of these cases involved the ethmoid sinuses either as a primary or by local invasion, and of these documented cases only one other has been found to solely involve the ethmoid sinus. We present a case of primary ethmoid sinus chordoma treated by wide local surgical excision and present a review of the literature with regard to prevalence and treatment rationale.