<p>Ewing sarcoma is a common, aggressive and highly metastasizing bone and soft tissues tumor that most frequently affects children, adolescents and young adults and leads to death. Despite advances over the last four decades that have improved the 5-year survival rate, the recurrence rate remains high. Autophagy plays a role in quality control by eliminating toxic cellular components under normal conditions, maintaining cell viability and establishing cellular adaptability under stressful conditions. The studies highlight autophagy as a tumor promoter or tumor suppressor and hence caution should be maintained when considering it as a therapeutic target. The dual role of autophagy as a cytoprotective and cytotoxic mechanism underscores its significance in cancer. One of the hallmarks of cancer is dysregulated metabolism in which the biosynthetic demands of highly proliferating malignant cells necessitate the rewiring of metabolic pathways. Research has shown the significance of amino acids, glucose and lipid metabolism in the development of cancer. Despite much research on Ewing sarcoma, there are still many unanswered questions and difficulties regarding the functions and outcomes of autophagy and metabolism in the treatment. This minireview aims to investigate additional information and strategies for targeting autophagy and metabolism together in the treatment of Ewing sarcoma.</p>