Aim: Sturge weber syndrome (SWS) is a sporadic congenital neurocutaneous disorder. It belongs to phakomatoses group of disorders. It is basically combinations of manifestations such as port-wine stains (PWS) of the face, ipsilateral leptomeningeal angioma (cerebral malformations and tumours), glaucoma, seizures and mental retardation. Sturge weber syndrome type II have no evidence of brain involvement though it shows a vascular malformation on the face and the possibility of glaucoma. We hereby delineate a case of a female, reported with PWS and diagnosed as Type II Sturge weber syndrome. Case Report: We reported a case of a 35-year-old female who had a diffused swelling on the right maxillary region corresponding to the distribution of the cutaneous port-wine stain and glaucoma of the right eye. Positive manifestations with respect to skin, eyes and oral cavity along with negative radiographic findings, we arrived to the diagnosis of Type II Sturge weber syndrome. Discussion: Differential diagnosis of SWS includes Rendu Osler Weber syndrome, Von Hippel Lindau Disease and Maffucci Syndrome. Diagnosis is made only on the basis of clinical and imaging features. Conclusion: Dental procedures are associated with increased possibility of hemorrhage in sturge weber syndrome. Thus, doing treatment in such patients is a difficult task for oral health care practitioners. Therefore the intent of the presented case is to keep in background a clinical scenario of similar pattern, if observed, will need to be diagnosed with utmost caution prior to any dental procedure.