A case of primary chordoma of the maxillary sinus is presented. The embryology of the notochord is reviewed and it is suggested that the frequently used adjective “ectopic” is a misnomer. The literature of primary paranasal sinus chordomas is reviewed and the clini- copathological features described. It is emphasized that these lesions can mimic other be-nign and malignant processes both clinically, radiographically and histologically. The treatment and prognosis is more favorable than the more common craniocervical chor-doma.