The distal humerus is a rare site for primary bone sarcoma. Limb salvage surgery with good oncological margins is possible, as the brachialis muscle acts as an anatomical barrier to the anterior part of the distal humerus and protects the major neurovascular structures. The ulnar nerve is in close proximity to the medial epicondyle and should be evaluated carefully for direct tumour infiltration. The modularity of distal humerus endoprostheses, which provides a stable and functional elbow reconstruction, results in good outcomes.