Congenital malignant rhabdoid tumour of the gum margin

The case of an infant born with a large polypoid tumour arising from the mouth is described. The tumour had the histological, immunohistochemical and ultrastructural phenotype of an extrarenal malignant rhabdoid tumour and followed an aggressive clinical course. This is one of the few reported cases of malignant rhabdoid tumour to present at birth. The oral tumour was associated with a mass in the posterior cranial fossa. This was most likely to be a simultaneous second primary tumour.