Childhood-onset Systemic Lupus Erythematosus (SLE) has an incidence of 0.3-0.9/100,000 children. It is very rare to find a case of childhood-onset SLE before 5 years of age. We describe a case of a 4-year-old female child, from a low-socioeconomic background who was diagnosed with SLE based on a typical rash, oral ulcer, positive laboratory parameters such as ANA, anti-ds DNA, anti-nucleosome, anti-histone antibodies, and positive lupus anticoagulants. There was an involvement of each system with considerable signs and symptoms including lupus nephritis, a cardiological complication such as Libman-Sack endocarditis, and neurological manifestations showing diffuse cerebral atrophy. The child was also positive for parameters linking to the presence of Macrophage Activation Syndrome MAS). The hypercoagulable state of SLE has been linked to more central nervous system involvement, cardiovascular dysfunction, renal complication, and hyponatremia as indicated in our case making it more riveting.