We are yielding a case of recurring melanotic neuroectodermal tumor of infancy situated in the premaxilla, with a very quick evolution, haemorrhage, and which has precociously recurred after surgical removal. This tumor is studied on the paraclinical (CT scan, M.I.R.) and on the histological hand (immunohistochemical and ultra-microscopy). This observation is then compared with those of literature in which we find about 200 cases. The neurocristopathic histogenesis is actually doing the unanimity. The prognosis must now be quite reserved because of the recurrences (one case for six) and the possibility of malignant forms, some with metastasis.