Proximal humerus chondrosarcoma is a rare localization of the common primary malignant cartilaginous tumor. Management is based on oncological surgical excision because of the inherent resistance to chemotherapy and radiation therapy. The most important prognostic factors with great impact on treatment strategy are histological grading and localization of the tumor. Proximal humerus chondrosarcoma carries a slightly better prognosis, especially for the low and moderate grade tumors. We present the case of a young patient with proximal humerus chondrosarcoma surgically managed in our Department, with the purpose of underlining the challenges posed by this localization for oncological excision and reconstruction but also the importance of histological grading for the treatment algorithm.