A rare case of melanotic neuroectodermal tumour of infancy affecting the maxilla is reported. These are rare, mainly benign neoplasms that present during infancy. Prompt surgery and rehabilitation using partial dentures are essential to minimise the effects of the deformity. The integrated surgical and prosthodontic management needed to attain good restoration of aesthetic and masticatory function in a child are emphasised. Additional partial dentures need to be made that accommodate the changes in the dental arch that occur as the child grows.