Stewart's malignant centrofacial granuloma is a rare affection. The characteristic clinical features are extensive ulceronecrotic processes affecting the upper respiratory tract, these being always unilateral, with no lymph node adenopathy spread and no visceral dissemination (confirmed in all cases in which an autopsy was conducted). There are no specific biological changes that could supply objective confirmation of the affection. The histological appearance is that of a granuloma with non-specific inflammatory signs, but having a pseudo-malignant appearance in some cases which is difficult to interpret. The true etiopathogenic nature of the lesions remains obscure. The most admitted current hypothesis is that Stewart's malignant granuloma is an immune response, in a granulomatous mode, to an antigenic stimulation, the antigen being unknown.