The melanotic neuroectodermal tumour of infancy is a rare, pigmented neoplasm that most frequently arises from the anterior maxillary alveolar ridge. It presents in the first few months of life, and usually follows a benign course. A classical case is presented and the clinical, radiological and pathological characteristics are discussed. The importance of early conservative surgical excision is emphasized with, in general, a good prognosis. This tumour, which many otolaryngologists may not be familiar with, should be included in the differential diagnosis of head and neck neoplasms in infants and young children.