Sturge-Weber syndrome or encephalotrigeminal angiomatosis is an uncommon neurocutaneous syndrome that manifests with vascular malformations involving the brain, eye and skin; Severe cases present with refractory seizures, sometimes requiring major surgery such as hemispherectomy. Most of the times, some degree of mental retardation and behavioral problems are associated, requiring use of psychotropic medication and other contention strategies. This report describes the case of a 19-year-old boy who was submitted to a left hemispherectomy by the age of one, and was still able to successfully complete basic education. He started presenting severe behavioral problems, with aggressive outbursts, by the beginning of adulthood, having been committed to psychiatry ward. By the age of 22, the patient finds himself calm and functional considering his limitations, with no need for hospital admission for 2 years.

To describe a clinical case whilst reviewing literature concerning this matter.

Case report with complete clinical history and medical data. Non-systematic review of PubMed database under the terms “Sturge-Weber disease”, “Hemisferectomy”, “behavioral disorder due to organic causes”, “post hemisferectomy out-comes”.

Although presenting with severe arteriovenous malformation, refractory epilepsy and left hemispherectomy, the patient was able to conclude basic instruction; He has lived with his family until the age of 19, when he started displaying disruptive behaviour; after 3 hospital admissions and perfecting psychotropic drugs treatment, the patient was admitted to an institution for rare diseases patients Since then he has experienced a calm and functional life, with trained professionals who can offer the non-pharmacological approaches he needs.