Background: Systemic lupus erythematosus (SLE) is a chronic acquired autoimmune multisystemic disease. Homocysteine (HCys) plasma levels increase frequently in children with (SLE). Hyperhomocysteinemia (HHCys) represents a risk factor for many morbidities in SLE patients, such as the formation of atherosclerotic plaques and atherothrombotic events. Also, HHCys was found to be associated with dyslipidemia and vitamin D deficiency in these patients. This study aimed at measurement of the level of HCys in children with SLE. Methods: A retrospective case-control study was carried out in Pediatric Department, Zagazig University Hospital during the period from December 2016 to January 2018. 35 patients diagnosed as SLE, and 35 healthy controls were included in the study. All participants were subjected to; Full clinical history taking, clinical examination and investigations as complete blood count (CBC), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), antinuclear antibodies (ANA), renal and liver functions, lipid profile, Vitamin D level and serum homocysteine level. Results: Serum homocysteine level was significantly higher in children with SLE than control group (P<0.05).Dyslipidemia was found to be frequent inchildren with SLE and related to elevated homocysteine level which both represented cardiovascular risk factors in these patients.Also children with SLE showed difficiency in vitamin D level. Conclusions:The present study proved that elevated plasma homocystiene level was frequent in children with SLE which may help in predicting cardiovascular risk in these patients. Keywords: Systemic lupus erythematosus (SLE)-Homocysteine-Vitamin D-children-Dyslipemia