The purpose is to detect which surgical techniques produce more satisfactory results in isolated pediatric primary Ewing sarcomas of the orbital pyramid. In addition to this primary purpose, initial symptoms in order of prevalence, behavioral traits of the tumors mentioned above, and diagnostic challenges have been discussed. By conducting an extensive search through the central databases of PubMed/Medline, Web of Science, and Cochrane, we have identified a limited number of 50 cases involving pediatric primary Ewing sarcomas. These cases encompass tumors originating within the orbital cavity and those extending from adjacent bony structures or paranasal sinuses . The exclusion criteria comprised adults aged 19 years or older, cases of primitive neuroectodermal tumors or neuroblastoma, and individuals diagnosed through autopsy. The mean age was recorded as 10.26 years. The male-to-female ratio was 1.45:1. The swelling and exophthalmos were the most prevalent presentation, followed by nasal-related signs and symptoms such as blockage or discharges, whether they were clear, turbid, or bloody rhinorrhea. The involvement of the right and left sides displayed no significant disparity. Among the infiltration sites, the orbital roof presented the highest level of complexity, whereas the erosion levels of the floor, lateral, and medial walls were nearly equivalent. The tumor infiltrated the whole of the orbital cavity in just two cases. The prevailing methods employed by reconstructive surgeons throughout the 74-year review consisted primarily of frontotemporal craniotomy, with or without orbitozygomatic osteotomy, followed by anterior orbitotomy. Although rare, the primary Ewing sarcoma of the orbit tends to present with proptosis or prominence and affects the superolateral walls of the orbit. The pediatric population may not express pain; exophthalmos and tissue swelling were the most typical manifestations. Furthermore, based on the relative prevalence of orbital roof involvement, more frontotemporal surgical approaches were documented in the literature. Level of Evidence: III. Type of Study: Systematic review.