<p><strong>Introduction</strong>: Sturge-Weber syndrome (SWS) is a rare condition characterized by facial capillary malformation, involves ocular, neurological, and cutaneous alterations. Associated with unilateral characteristic port-wine stains, gingival growth and purple-red coloration. <strong>Aim</strong>: his case aims to report dental treatment challenges in patients with SWS and importance of oral health maintenance in these individuals. <strong>Case report</strong>: a 20-year-old woman with an established diagnosis of SWS, presented bad breath and spontaneous gingival bleeding, with gingival growth and reddish-purple spots spread to labial and alveolar mucosa, tongue, and palate. Conditioning of the patient’s oral environment by supra and subgingival scraping, dental unit extraction was performed. A conservative treatment plan was adopted for management adequacy of oral environment owing to possible complications inherent to the condition. <strong>Conclusion</strong>: it is important to emphasize the importance of dental surgeon’s performance in relation to a multidisciplinary health team, as well as cooperation of patient, to obtain better results from the proposed therapy</p>