Congenital primary primitive neuroectodermal tumor of the orbit in a newborn

Introduction: Primitive neuroectodermal tumors arise from the progenitor cells of the neural crest, in the central nervous system or other peripheral locations. Case presentation: We report a rare case of a congenital malignant tumor, diagnosed as a primary orbital primitive neuroectodermal tumor on histopathological examination. Conclusion: Multidisciplinary management with adjuvant chemotherapy needed for the management of these cases.