A case of melanotic neuroectodermal tumour of infancy is decribed. The pre-operative diagnosis was made on cytological material obtained by fine needle aspiration. The patient was a three-month-old male infant with a rapidly growing maxillary tumour mass that also involved the pterygomaxillary fossae and the floor of the orbit. In addition to the typical clinical presentation, the cytology is also distinctive showing a dual population of small neuroblastic cells and large melanin-containing epithelial cells. Histological, immunohistochemical and electron microscopic examination of the excised mass confirmed the initial diagnosis. The pre-operative distinction of this tumour from other small round cell tumours of infancy (rhabdomyosarcoma, neuroblastoma, melanoma and lymphoma), is essential in order to plan the most complete resection therefore reducing the possibilities of tumour recurrence. This tumour belongs to a field of pathology with which many otolaryngologists may not be familiar.
