OBJECTIVE: To characterize neurological involvement in juvenile systemic lupus erythe-matosus. METHOD: The charts of all patients with the diagnosis of systemic lupus erythematosus before the age of 16 years, followed at the Rheumatology Unit of Pequeno Príncipe Hospital, from January 1992 to January 2006, were retrospectively reviewed, highlighting neuropsychiatric aspects. RESULTS: Forty-seven patients were included. Neuropsychiatric syndromes were found 29 (61.7%): seizures (17 / 36.2%), intractable headache (7 / 14.9%), mood disorders (5 / 10.6%), cerebrovascular disease (4 / 8.5%), acute confusional state (3 / 6.4%), aseptic meningitis (3 / 6.4%), psychosis (3 / 6.4%), chorea (3 / 6.4%), Guillain-Barré syndrome (2 / 4.3%) and cranial neuropathy (1 / 2.1%). Morbidity indexes (SLEDAI and SLICC) were higher among patients with neuropsychiatric manifestations (p<0.05). CONCLUSION: Neuropsychiatric syndromes are frequent, and add significant morbidity to juvenile systemic lupus erythematosus.