Perforating pseudoxanthoma elasticum is a localized, cutaneous disorder found mostly in obese, multiparous middle-aged women. It is characterized clinically by a slowly enlarging, yellowish, well-defined reticulated plaque usually in the periumbilical area with keratotic papules scattered on its surface, and histologically by transepidermal elimination of altered basophilic calcified elastic fibers, which are short, fragmented, and curled, in the middle portion of the reticular dermis. We present a case of a 36 year-old female who had a skin lesion, which showed chracteristic features consistent with perforating pseudoxanthoma elasticum in her neck region but not in the periumbilical area. (Korean J Dermatol 2000;38(3)：424~426)