Background: Pseudoxanthoma Elasticum (PXE) is a rare disease with autosomal recessive inheritance. Dysfunctional or absent ABCC6 results in low serum PPi, ectopiccalcification, obviously of elastic tissue, eyes, and blood vessels. Clinical signs of PXE usually begin in early childhood, and skin changes are frequently the earliest sign of PXE. These skin lesions tend to progress slowly. PXE is related to the risk of blindness and peripheral vascular compromise. We present a case of a male patient who is possiblydiagnosed Pseudoxanthoma Elasticum at Hue Central Hospital. Case report: A 20 - year - old male patient came to our clinic to check his skin lesions. According to him, the lesions appeared when he was 13 years old. Small flesh-colored papules appeared sparsely first at theperiumbilicaland abdominal areas with the changing size from 1-5mm. Gradually, such papular lesions coalesced to form bigger papules or plaques, and new skin lesions appeared symmetrically on abdomen, chest, twoarmpits, two arms and two groins. These lesions progressed slowly in years with no signs of inflammation or ulceration. Moreover, the patientdid not feel painful or itchy sensations. After conducting the skin biopsy, the patient was expected to have Pseudoxanthoma Elasticum (PXE) disease, and he was transferred to the related departments for examination. Conclusion: The recognition of PXE is extremely important because it is associated with the risk of blindness and peripheral vascular compromise. The faster the detection and setting of diagnosis are made, the more extreme the preventive measures and the surveillance to prevent and control the progression of the disease are applied.