Childhood-onset systemic lupus erythematosus (cSLE) is a severe, chronic, multi-organ and systemic autoimmune disorder characterized by inflammatory and autoimmune reaction in several organs. The occurrence of SLE in children is very rare. About 20% of all SLE cases are diagnosed during the first 2 decades of life and the disease is extremely rare before age of 5 years. In this case report, we explore a 3-years-old girl with SLE that symptoms like Primary Herpetic Gingivostomatitis. Early diagnosis lead to proper treatment of the disease and it is important to decrease oral complications in children. Diagnosis could be improved by introduce new cases to provide valuable information for dentists based on diagnostic criteria, therapeutic steps and complication of treatment of SLE in Children. Therefore, it could be concluded that dentists involved in pediatric dentistry should consider and work out on the clinical signs of SLE in children with history of oral herpesvirus infection.