Emery-Dreifuss muscular dystrophy is a rare form of muscular dystrophy. In the present study we present two patients with Emery-Dreifuss muscular dystrophy and severe upper extremity contractures treated successfully with contracture release and musculotendinous lengthenings. For each of these patients a chart review was carried out and surgical technique reviewed. Patient 1 demonstrated elbow flexion contractures of 65° (right) and 60° (left) preoperatively and ∼45° (right) and 20° (left) postoperatively. Patient 2 demonstrated flexion contractures of 75° bilaterally preoperatively and 45° bilaterally postoperatively. For more severe elbow contractures, surgical treatment resulted in improved range of motion without a significant sacrifice of strength, allowing these patients to remain independent.