Hydatid bone disease is caused by the Echinococcus granulosus worm. The location of the disease in the humerus is infrequent. No reports of hydatid disease in the entire humerus have been found in the bibliography. We present one case of primary hydatid bone disease affecting the entire humerus, which was treated with radical resection and total endoprosthesis of the humerus. A 24-year-old patient presented with a diaphyseal humerus fracture. The x-rays and MRI showed an oblique fracture and heterogeneous osteolytic and multiloculated images along the entire humerus. The biopsy resulted in hydatid cysts. Oncological resection of the humerus and total replacement of the same with a non-conventional prosthesis designed for the patient was performed. Total humerus hydatidosis is infrequent, and there is scarce information about its treatment. The local resection plus graft and osteosynthesis conserving the bone has shown complications and recurrences. For healing, a radical procedure is necessary. Although functional results may be limited. Copyright © 2019 The Authors. Published by Elsevier Ltd.. All rights reserved.