Periosteal lesions of the ulna diaphysis are rare, include a wide spectrum of tumors, and may cause considerable diagnostic problems. Surgical treatment may vary widely, based on an accurate diagnosis. We present the case of a periosteal, extraskeletal low grade myxoid chondrosarcoma of the ulna diaphysis. The surgical therapy included an en-bloc resection with allograft reconstruction. The patient showed a favorable outcome. Careful preoperative evaluation and planning are imperative to obtain a satisfactory oncological and functional outcome, especially with uncommon tumor presentations at rare locations.