Pseudoxanthoma elasticum (PXE) is a rare autosomal recessive inherited multisystem disease caused by mutation of the ABCC6 gene and characterized by progressive mineralization and fragmentation of elastic fibers affecting different tissues, mainly the skin, the eye with calcification of Bruch’s membrane, and the cardiovascular system with premature atherosclerosis. Cutaneous lesions, which often represent the first manifestations of the disease, appear as soft, small, yellowish asymptomatic papules confluent in plaques with cobblestone aspect. Dermatoscopy of PXE shows the presence of multiple irregular yellowish areas, alternating with prominent linear vessels, that may coalesce into parallel strands and/or streaks. Dermatoscopy is a valuable tool for a prompt diagnosis of PXE, so as to address the patient toward the appropriate workup.