Constriction band syndrome is an uncommon congenital abnormality with multiple disfiguring and disabling manifestations. Early amnion rupture with subsequent entanglement of fetal parts by amniotic strands is currently the primary theory for the development of this syndrome. Management of constriction band syndrome is focused on improving function and development while providing a more acceptable esthetic appearance. The treatment should be tailored to the individual. Timing of repair and surgical planning are important to provide the best functional results for affected hands.