A rare syndrome, first described in 1938 by FREEMAN and SHELDON, two English doctors, is discussed. The condition affects the face and both hands to a degree that no useful function is possible. The characteristic malformations are described in detail. They are caused by a soft tissue disorder and no bony involvement has been found so far. Therefore, the term "Cranio-carpo-tarsal dystrophy" is misleading. Conservative treatment by dynamic splints has brought very little improvement.