Abstract
Melanotic neuroectodermal tumors of infancy (MNTI) are a rare type of benign tumor that affects the head and neck region. MNTI represents (68–80%) of the tumors in the maxillary region. This tumor is usually located in the alveolar crest, skull, mandible, and brain. Although this tumor is considered benign, it can grow rapidly, with a high risk of recurrence and interference with functions of infancy, such as feeding and breathing. It is also frequently harmful to the surrounding soft and hard tissues or adjacent sensitive vital structures. This study aimed to review the pathological, clinical presentation, and treatment of melanotic neuroectodermal tumors in infancy and the role of dentists in these cases.