Adamantinoma-like Ewing sarcoma (ALES) is a rare variant of the Ewing sarcoma family characterized by epithelial differentiation in a tumor harboring molecular features of conventional Ewing sarcoma. From a pathological standpoint, it poses a significant diagnostic challenge due to its overlapping morphology with basaloid carcinomas and other small round blue cell tumors. Histologically, ALES demonstrates nests and sheets of small round to basaloid cells with peripheral palisading, rosette-like structures, and occasional squamoid differentiation. Immunohistochemically, the tumor exhibits a distinctive biphenotypic profile with diffuse CD99 expression along with cytokeratin and p40/p63 positivity. The presence of EWSR1 gene rearrangement or other FET-ETS fusions confirms the diagnosis. Accurate recognition is essential, as misinterpretation may lead to inappropriate management strategies.