Ewing Sarcoma (ES) is mainly diagnosed during the second decade of life. It often compromises the appendicular skeleton but also can affect other bones. A surgical procedure is necessary despite its good response to chemotherapeutics and radiotherapy. When planning limb-sparing procedures for the young patient, the main challenges are bone size, growth remaining, and physical demands.

Several techniques have been proposed. However, there is no gold standard procedure because many factors must be considered, and each case should be individualized. Also, the resources available change from one medical center to another and every surgeon has to deal with a different reality.

This article presents one case of a 5-year-old male patient diagnosed with ES in the diaphyseal region of the humerus. It reviews the possible surgical options for humeral reconstruction in young children with ES and discusses the challenges that surgeons might have overcome.