Sturge weber syndrome or encephalotrigeminal angiomatosis which is a rare sporadic, nonhereditary, neurocutaneous condition affecting other multiple system of body which includes CNS, ophthalmic, oral, endocrine, vascular, ENT. oral manifestation is seen in 40% cases. In this case 12/F patient presented with port wine stain involving left side of face involving maxillary division of trigeminal nerve, gingival over growth involving left maxilla and cone beam computed tomography suggestive hypertrophied lining of maxillary sinus associate with Osteohypertrophy of left maxilla. Surgical excision of lesion was done under GA & histopathology was suggestive of capillary haemangioma. In this paper a special emphasis is placed on other manifestation of Sturge weber syndrome and their management along with oral manifestation.