A 3 month old Omani boy developed fever with skin rash. He was taken to many local health centers, got treatment but without any lasting benefit. At the age of eight months he developed status epilepticus and was brought to Royal Hospital, Muscat, Oman, for further management. He was thoroughly investigated and diagnosed as infantile Systemic Lupus Erythmatosis (iSLE). He responded very well to the treatment and is now in complete remission. iSLE is a rare disorder; this made us to search and review the experience of the experts worldwide.