INTRODUCTIONPseudoxanthoma elasticum (PXE) is a rare autosomal recessive (AR) genetic disorder caused by different mutations in the ABCC6/MRP6 gene located on the chromosome 16p13.1, which encodes for a transmembrane protein involved in transport and tissue mineralization homeostasis.1 As a result of diverse gene mutations, dystrophic calcification of elastic tissues occurs, primarily through calcium deposits at cutaneous, ocular, and cardiovascular level, although other systems with elastic tissues may be affected. Its estimated global prevalence of 1:25,000 to 1:100,000, with a slight female predominance (2:1) and no major racial or ethnic predilection.2The earliest manifestations of PXE typically arise in the skin during the second to third decade of life, with the appearance of small, isolated yellowish papules that coalesce into reticulated plaques on flexural areas, giving a cobblestone-like appearance.3In some cases, mucosae may be involved. Ocular and vascular involvement develop at final stages.4,5 Currently, a combination of clinical and histopathological criteria is used to establish the diagnosis (Table 1).6 There is no curative treatment for PXE.7 Management is symptomatic and multidisciplinary, including monitoring and treatment of complications. We describe a case of a patient presenting with nonfamilial PXE with classical manifestations on the skin. We emphasize the significance of clinical and histopathological examination in the differential diagnosis, with a review of the current therapeutic options.