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Round cells are unified by morphology, differing widely by histogenesis. It generally contains sheets of round cells which are small having large nucleus, scant cytoplasm, hyperchromatic or vesicular nucleus. They are mostly highly undifferentiated neoplasms composed of a monotonous population of round cells with high Nuclear: Cytoplasm ratio [1]. These are also called round blue cell tumors as the cells appear blue due to presence of large hyperchromatic nuclei and a thin rim of cytoplasm [2]. Round cell tumors, although rare in oral cavity; but imposes a diagnostic challenge for pathologists due to their undifferentiated or primitive character. Differentiating these from others still remains an enigma [3]. This group of tumor includes entities such as peripheral neuroectodermal tumor, rhabdomyosarcoma, synovial sarcoma, non-Hodgkin’s lymphoma, neuroblastoma, hepatoblastoma, Wilms’ tumor, and desmoplastic small round cell tumor. These round cell tumors are characterized by typical histological pattern, immunohistochemical, and electron microscopic features that can help in differential diagnosis [4].