A 50-year-old woman presented with a 2-year history of a yellowish plaque studded with red brown keratotic papules in the periumbilical region. Histopathological examination from the yellow plaque showed curled and granular elastic fibers in the mid and lower dermis. Histopathological examination from a keratotic papule showed pathological elastic fibers and dense chronic inflammatory cells around areas of perforation. Clinicopathological correlation established periumbilical perforating pseudoxanthoma elasticum as the final diagnosis.

Median raphe cysts are rare congenital lesions ofthe male genitalia that occur as a result of alteredembryologic development. We report two such casesof median raphe cysts in the pediatric age group. Inaddition, we review the literature.