Ameloblastoma is an odontogenic neoplasm of enamel organ type tissue which does not undergo transformation to the point of enamel formation. We present the second case in the English literature of maxillary ameloblastoma that presented with nasal obstruction and rhinorrhoea, and the first to be excised using a combined maxillotomy and endoscopic ethmoidectomy. The patient had no previous dental history. The unusual presenting symptoms, as well as the highly destructive nature of these lesions when arising in the maxilla, make them worthy of consideration in the differential diagnosis of nasal and maxillary masses. We discuss the clinical features, pathology and management of these lesions and review the literature.