The Freeman-Sheldon syndrome is a rare symptom complex. The hand has flexion contracture and ulnar deviation of the middle finger as well as flexion and adduction contracture of the thumb. The clinical and genetic findings in 11 cases and the results of treatment of six hands are reported. Deformity of the thumb is foremost in contributing to the functional impairment of the hand in determining the mode of treatment. Several different plans of management are available: Z-plasties, muscle transposition, capsulotomies, skin flaps. The treatment should be individualized.