Pseudoxanthoma elasticum is a rare, heritable, systemic disease of connective tissue characterized by degeneration of elastic fiber and mainly affects the skin, eye and blood vessels. We report a case of pseudoxanthoma elasticum mimicking cutis laxa. She had a generalized laxity of the skin for about 30 years. There were similar skin lesions in her sister. No other systemic involvement was found. Histopathologic examination revealed clumping, fragmentation and calcification of elastic fibers in the dermis. (Korean J Dermatol 2000;38(2)：227~230)