A-14-year-old Japanese boy with Klippel-Trénunay-Weber syndrome is reported. The major clinical features of this patient were a portwine stain nevus, varicose veins and the hypertrophy of the bones and the soft tissue of his left leg. In addition, numerous angiokeratomas, a pyogenic granuloma and Polydactyly of the affected limb were also seen. A skin biopsy of the portwine stain nevus revealed the histological features of the portwine stain nevus to consist of hemangiomas associated with lymphangiomas. No evidence of an A-V shunt was found by angiography of the affected limb. The pyogenic granuloma was successfully treated by CO2 laser therapy without any bleeding.
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