The melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that affects mainly the anterior aspect of the maxilla during the first year of life. Although the MNTI is accepted as a benign tumor, it often grows rapidly, can invade bone marrow diffusely, and his recurrence rates ranges from 10% to 20%. Clinical features of the tumor are its blue or black appearance and, occasionally, an ulcerated surface. This lesion was reported under a variety of names that reflects the controversy about its origin and histogenesis. Recent studies have made the neural crest origin widely accepted. The aim of this study is to report a case of a MNTI affecting the anterior maxilla of a 5-month-old female child. The patient presented to the department of Oral and Maxillofacial Surgery of the Pedro Ernesto University Hospital in September 2010 for evaluation of a relatively fast growing mass on her upper lip and alveolar ridge. The lesion was first noticed buy her mother one month before. A incisional biopsy was performed under general anesthesia. Subsequently the microscopic examination of the specimen was consistent with MNTI. Two weeks after the first procedure the patient returned to the operating room and a conservative surgical excision of the tumor was performed. The patient was followed up for 9 months so far and presents with no sign of recurrence and good bone healing.

Conflict of interest: None declared.