Ewing sarcoma (ES) rarely involves acral regions as the foot. A 4-year-old white female presented with a growing and painful violaceous nodular lesion at the fourth right toe, which appeared 4 months prior to the consultation. MRI reported a nonspecific soft tissue tumor with intense homogeneous contrast uptake. Histopathological examination revealed CD99 positive, small, round, blue cells. EWS-FLI1 fusion gene translocation was detected, consistent with ES. In this report, the authors show the notable response to neoadjuvant chemotherapy of an infrequent foot phalangeal ES.