Objectives <p>To assess the clinical characteristics and outcomes of children with neuropsychiatric systemic lupus erythematosus (NPSLE).</p> Methods <p>A retrospective analysis of demographic, clinical and laboratory data of Juvenile onset systemic lupus erythematosus (jSLE) patients managed at a tertiary rheumatology clinic over 30 y was performed. Results were expressed as proportions, mean with standard deviation and median. Comparative analyses and multivariate logistic regression were used to identify independent predictors of NPSLE.</p> Results <p>Among 338 jSLE patients, 25.1% patients had NP manifestations. The mean age at presentation was 13.86 y. Seizures were the most common manifestation (62.3%), followed by psychosis (17.6%), mood disorders (14.1%), neuropathies (8.2%), chorea (4.7%) and transverse myelitis (3.5%). On univariate analysis, serositis, nephritis, constitutional symptoms, higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores and lower anti-phospholipid antibodies levels were associated with NPSLE; however, these associations were not significant on multivariate analysis adjusted for age and sex. Among 69 patients with follow-up, (median 55 mo), 76.8% had no recurrences. Recurrent NP manifestations occurred in 10.1%. Residual damage was noted in 4 patients (hearing loss, foot drop, and quadriparesis). Five patients died (2 due to status epilepticus, 1 due to infection and 2 due to non-neurological causes).</p> Conclusions <p>NP manifestations occur in a quarter of patients with jSLE. Recurrences and significant morbidity is seen in 10% of patients.</p>