Myeloid sarcoma (MS) occurs when immature myeloid cells settle in the extra medullary area. It may be associated with acute myeloblastic leukemia (AML) or it may be alone and then develop AML. The orbit is one of the places where myeloid sarcoma is common in childhood. In this case report, we present a case of orbital myeloid sarcoma living in Africa, presenting with advanced proptosis and vision loss, whose proptosis improved dramatically with chemotherapy and vision returned.