Tehran University of Medical Sciences Acta Medica Iranica 0044-6025 57 2 2019 03 12 143 146 EN Souhir Khemiri Department of Medical Oncology, CHU Habib Bourguiba, Sfax, Tunisia. Jihene Feki Department of Medical Oncology, CHU Habib Bourguiba, Sfax, Tunisia. Afef Khanfir Department of Medical Oncology, CHU Habib Bourguiba, Sfax, Tunisia. Mohamed Abdelmoula Department of Maxillofacial Surgery, CHU Habib Bourguiba, Sfax, Tunisia. Mounir Frikha Department of Medical Oncology, CHU Habib Bourguiba, Sfax, Tunisia. 2018 01 31 2018 11 05 Melanotic neuroectodermal tumor of infancy (MNTI) is a rare and distinctive neoplasm of early infancy with rapid expansile growth and a high rate of recurrences. Most commonly the lesion affects the maxilla of infants during the first year of life, but it may also occur in the mandible, skull, brain, epididymis, and other rare locations. Common treatment methods’ include surgical excision and resection of the tumor. The aim of this article was to show the diagnosis and treatment of a 7-month-old patient with melanotic neuroectodermal tumor occurred in the anterior mandible and to demonstrate the effectiveness of the neoadjuvant chemotherapy. https://acta.tums.ac.ir/index.php/acta/article/view/7005 https://acta.tums.ac.ir/index.php/acta/article/download/7005/5227