Objective: The aim of the present study was to determine neurocognitive profile in Children with Systemic (SLE) and Neuropsychiatric Lupus (NPSLE) describing differences in the seven cognitive areas proposed by the ACR (attention, memory, visuospatial processing, language, problem solving, processing speed, and executive function) between children with SLE and those with NPSLE and to understand the level of cognitive deterioration in children afflicted by SLE with and without neuropsychiatric changes. Method:Children with SLE and with NPSLE were evaluated using the Wechsler Intelligence Scale for Children (WISC-IV) and some selected subtests of the Neuropsychological Assessment for Children (NAC), which allowed us to measure the 7 cognitive areas proposed by the ACR. Both, SLE and NPSLE children performances were compared to scores obtained by children without any affection. Results:The area's most affected in the NPSLE group were attention, working memory, processing speed, memory, and visuospatial ability; in the SLE group, the area's most affected were processing speed, visuospatial ability, planning, and auditory memory.Conclusion: Based on our findings, it is concluded that frequently in both groups cognitive decline is present since early stages of illness, being more important in the NPSLE group. These deficiencies are heterogenous and with a multi-domain pattern.