Introduction/Objectives: Evaluate the most common clinical features of children and adolescents with neurocutaneous disease Sturge-Weber. Methods: The records of 28 patients who were diagnosed with Sturge-Weber disease at CENEP-HC-UFPR in the period 1970 to 2013 and collected data regarding the changes more commonly found in this condition were evaluated. Results: Of the 28 patients, 12 were male and 16 were female. The mean age at first visit was 63.3 months (1 to 163m). Three patients didn't have the typical hemangioma of the disease but had radiological changes compatible. In addition to injuries in face, lesions in trunk (7), neck (5), MMSS (3), MMII (4), dorse (1) and lumbar (3) were observed. About 28,5% of patients had a diagnosis of glaucoma. Epilepsy was present in 53.5% of patients, and of these, 40% refractory to anticonvulsant treatment. Headache was present in 21.4% of the sample, 33.3% related to seizures. Approximately 53,5% of patients had electrographic changes being 33.3% irritative activity. Cognitive impairment was found in 17.8% of patients and psychiatric disorders in 14.2%. Conclusion: Besides the skin changes, Sturge-Weber disease can course with comorbidities such as epilepsy, glaucoma, mental retardation and psychiatric disorders, which must be diagnosed and treated early.