Port-wine stains (PWS) usually occur unilaterally alongdermatomes supplied by the ophthalmic and maxillary division of trigeminal nerve. PWS could also be bilateral and extend to neck, extremities and other parts of the body or even absent.The Sturge-Weber syndrome (SWS) is a rare, sporadic, neurocutaneous disorder with classical presentations of facial PWS, vascular eye abnormalities, and leptomeningeal angiomatosis. Pyogenic granuloma is a reactive vascular lesion that often arises at sites of minor trauma. It can also occur within pre-existing vascular lesions such as PWS or arteriovenous malformations. In this case report, we present a 14-year-old Korean girl diagnosed with SWS presenting atypical generalized distribution of PWS with pyogenic granuloma and late-onset leptomeningeal angiomatosis.