Abstract
Summary Melanotic neuroectodermal tumour of infancy is a rare pigmented neoplasm of neural crest origin that usually affects infants. The majority of reported cases have occurred within the maxilla. We describe a case that arose within the mandible of a 1-week-old boy. The diagnosis is established by histological examination, which classically demonstrates two distinct cell populations: neuroblast-like small round cells and pigment laden epithelioid cells. Treatment consisted of hemimandibulectomy with primary reconstruction using autologous costochondral rib graft. Although controversial, we advocate this method of reconstruction in infants with extensive mandibular continuity defects. In the present case the histological features varied on the initial biopsy and resection specimens, differing in exposure to chemotherapy which had apparently eradicated the small round cell population.
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