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The Sturge-Weber syndrome consists of unilateral port wine hemangioma of skin along the trigeminal distribution associated with an ipsilateral leptomeningeal angioma. Nevus of Ota (oculodermal melanosis) is a hamartoma of dermal melanocytes which presents as a blue or gray patch on the face and conjunctiva, which is congenital or acquired and is within the distribution of the branches of the trigeminal nerve. The coexistence of cutaneous hemangioma and pigmentary naevi is known as phakomatosis pigmentovascularis. We report the first case of phakomatosis pigmentovascularis in the Indian literature.