Small round cell tumors configure a heterogeneous group of malignant neoplasms that present clinical predominance in children and young adults. It encompasses a wide variety of undifferentiated primitive neoplasms which have in common the ability, with varying frequency, to present as a nondescript proliferation of round cells with high nuclear cytoplasmic ratio and hyperchromatic nuclei by light microscopy. Their differential diagnosis is influenced by the age of the patient, their site of occurrence, and slight degrees of differentiation exemplified by minor architectural and cytoplasmic features. All tumour types have special treatment modalities and the prognosis is dependent on the exact histological diagnosis confirmed by adjuvants like immunohistochemistry and Cytogenetics. This article reviews some of the most common small round cell tumors involving the head and neck region which includes Ewing’s sarcoma, rhabdomyosarcoma, neuroblastoma, and lymphomas.