Ewing sarcoma (ES) is the second most common bone cancer in children and can arise from any bone or, in rare cases, from soft tissues, the latter being referred to as extra-osseous Ewing sarcoma (EES). The management of ES is multidisciplinary, involving chemotherapy, surgery, and radiotherapy, with surgery (R0 resection) serving as the cornerstone of treatment. However, treatment poses significant challenges in low- and middle-income countries (LMICs) due to limited resources, including the frequent absence of facilities capable of providing advanced diagnostic tools such as scintigraphy or radiotherapy. Additional barriers include the prevalence of advanced disease at diagnosis, comorbidities such as infectious or parasitic diseases, and malnutrition. The level of care available, as determined by the diagnostic and treatment options accessible at a given center, plays a critical role in determining patient outcomes. This review comprehensively addresses various aspects of the diagnostic pathway, TNM staging, and treatment options, including supportive care. Furthermore, it highlights the importance of ethical considerations and palliative measures, ensuring a holistic approach to managing Ewing sarcoma in resource-constrained settings.