Melanotic neuroectodermal tumor of infancy is a rare，usually benign, pigmented congenital tumor of neuroectodermal which usually occurs in infants under one year of age. The maxilla is the most oftenly involves site. The mass which has a light blue or gray- black appearance in gross improves slowly. The tumor is benign but may invade bone marrow, very rare examples of malignant transformation have been reported. It was suggested to enlarge operation margin for avoid recurring. This review present the clinic feature, pathology and differential diagnosis, treatment and prognosis of the disease.
