Ectomesenchymal chondromyxoid tumour is a recently described rare entity found almost exclusively in the anterior dorsum of the tongue. We report an additional case, review the pertinent literature and explore the histogenesis of the lesion. The lesion has no discriminating clinical features and diagnosis is only established following histopathological examination including immunohistochemical assessment. The histogenesis of the lesion remains speculative and our study seems to discount myoepithelial cells as a potential source.