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We report a 7-month-old infant who had widespread port-wine stain involving the right side of the face, head and upper right limb. Nevus of Ota was also present on both frontal and upper eyelid regions. The trunk and extremities were almost totally covered by Mongolian spot. Other findings included hemihypertrophy of the right side of the face and right upper limb, and pigmentation of bilateral palpebral and scleral conjunctiva. Based on the clinical manifestations, the diagnosis of phakomatosis pigmentovascularis type IIb associated with Klippel-Trenaunay syndrome was made.