RESUMO
Introduction: Ewing's sarcoma is the second most common primary malignant bone tumor in children and adolescents, after osteosarcoma. It is a rare tumor, with the axial skeleton being the preferred site of development, followed by the long bones. Diagnosis is evoked by imaging and confirmed by histology. Treatment is based on intensive chemotherapy with local surgical treatment in operable forms, and in some cases, radiotherapy. There are only a few sporadic cases in the literature describing distal fibular localization. Case Report: We report the case of a 7-year-old child who presented with pain on the lateral aspect of the left ankle without local inflammatory signs. Radiological findings revealed a metaphyseal-diaphyseal osteolytic tissue process of the left fibula, and histology concluded that it was Ewing's sarcoma. We proceeded with neoadjuvant polychemotherapy followed by segmental resection of the distal fibula with an anteroexternal tibial rod, plus tibiotalar, and talocalcaneal arthrodesis without recourse to radiotherapy. Conclusion: The management of Ewing's sarcoma is constantly evolving. Its distal fibular location in a growing limb makes it even more difficult. It must be personalized, multidisciplinary, and carried out in specialized centers.