Periumbilical perforating pseudoxanthoma elasticum is a rare acquired disorder that usually occurs in obese, middle-aged, multiparous women. It is considered as a separate entity from the hereditary pseudoxanthoma elasticum due to lack of hereditary transmission and association with systemic disease. A 70-year-old multiparous woman presented with a 2-year history of a gradually enlarging, yellowish ulcerated plaque in the periumbilical region. The biopsy specimen showed irregularly altered elastic fibers with encrusted calcium salts that underwent transepidermal elimination. We report a rare case of periumbilical perforating pseudoxanthoma elasticum with no signs of hereditary pseudoxanthoma elasticum.