Juvenile localized scleroderma(JLS) is a fibrosing disease that affects the skin and subcutaneous tissue, and occurs before 16-year-old. Port wine stain(PWS) is a type of capillary vascular malformation composed of dilated blood vessels within the papillary and reticular dermis. A 22-year-old male patient visited our department for slightly erythematous depressed patch on his right thigh. Both the erythematous patch and the depression were found at childhood. He had no history of trauma, injection and other past medical disease. Physical examination revealed a solitary about 10cm x 10cm sized slightly erythematous depressed patch on his lateral side of right thigh. Histopathological examination showed relatively normal appearance of epidermis with hyperkeratosis, proliferation and dilatation of vessels with perivascular inflammatory infiltration in upper dermis and decreased number of adnexal structures. Mild atrophic subcutanous layer seemed to lie higher in the dermis and the collagen bundles in the dermis appeared thickened. Elastic fiber stain confirmed straightened elastic fibers. Masson trichrome stain revealed focally thickened collagen bundles. Based on these findings, a diagnosis was made as juvenile localized scleroderma occurring concomitantly with port wine stain. Considering its underestimated tendency, clinician needs to be concerned about cases of coexistence of JLS and PWS.