Abstract
Objective: To present a case of Sturge-Weber Syndrome (SSW) to instruct and alert professionals on the subject so that appropriate diagnosis and treatment can be performed when facing similar cases. Case report: Full-term newborn, without prenatal diagnosis, with presence of erythemato-macular lesions on face, limbs and chest at birth, associated with right upper and lower limb edema, eyelid edema and right cornea opaqueness. A clinical investigation was carried out and the suspicion of SSW was raised and complementary examinations were requested, which did not present any alterations. Patient evolved with glaucoma and convulsive crises, and continued in outpatient treatment. Final considerations: The case presented has great value for medicine since it is a rare disease that can bring serious consequences to the patient and his family dynamics. Thus, it is possible to conclude that medical practice requires high precision in diagnoses performed and knowledge of rare diseases so that there is quick diagnosis and in order to improve the prognosis of patients.