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.
Keywords: Ewing sarcoma; Primary primitive neuroectodermal tumor; newborn; round cell tumor.