Objective: We presented a case report, systemic lupus erythematosus (SLE) in a Malay boy. Interestingly, this case occurs in a boy, which is not so common because autoimmune disease usually occurs in female. Design: Case report. Methods: We highlighted a case of a boy with SLE who presented with clinical symptoms suggestive of SLE and fulfilled the criteria for SLE diagnosis. Results: The patient was successfully managed with antihypertensive, intravenous cyclophosphamide and oral prednisolone and respond well to the therapy. Conclusion: Systemic lupus erythematosus is a chronic autoimmune disease which rarely occurs in male. However we reported one such case which fulfilled the criteria for SLE. © 2017 Japan Health Sciences University & Japan International Cultural Exchange Foundation.