A case of congenital cutis laxa with advancedemphysematous changes in the lungs, documented by a lung biopsy, is reported. In addition, our patient had extensive changes in the ureters, presumably a part of the generalized disease. Studies of the serum level and urinary excretion of copper appeared to support the theory that in this disease there may be a disturbance in copper metabolism resulting in low levels of EIS with consequent increased destruction of elastic fibers. This disease is not always limited to the skin but is more often a systemic one producing lesions throughout the body, some of which are incompatible with life. A case of congenital cutis laxa with advancedemphysematous changes in the lungs, documented by a lung biopsy, is reported. In addition, our patient had extensive changes in the ureters, presumably a part of the generalized disease. Studies of the serum level and urinary excretion of copper appeared to support the theory that in this disease there may be a disturbance in copper metabolism resulting in low levels of EIS with consequent increased destruction of elastic fibers. This disease is not always limited to the skin but is more often a systemic one producing lesions throughout the body, some of which are incompatible with life.