Systemic lupus erythematosus (SLE) is an autoimmune disease that involves multiple organ systems. Musculoskeletal (MS) involvement occurs in either during the disease course in 70-95% of SLE patients or as an initial finding in nearly 50% of the cases. 1,2 MS involvement may vary from myalgia, arthralgia, non-erosive arthritis, myositis, tenosynovitis to contractures and avascular necrosis. 2 However, coexistence of sacroiliitis 3,4 or spondyloarthropathy (SpA) is really scarce. 5 In this article, we reported a patient presenting with juvenile SLE and SpA and discussed the clinical and laboratory findings by the literature review. To the best of our knowledge, this is the first pediatric case with coexistence of juvenile SLE and SpA. CASE REPORT A 16-year-old Syrian female patient was admitted to our hospital with low-back pain present for two months. She was born by vaginal delivery at 39 weeks as the second child of consanguineous parents (first cousins). She had initially presented to a local medical center with fever, rash, fatigue, oral ulcers and pancytopenia a year before. She was diagnosed with SLE. Prednisolone (1 mg/kg) and hydroxychloroquine therapy was administered. One year later, she was referred to our hospital due to complaints of low-back pain worsening in the morning and after a long resting period during the last two months. She also suffered from morning stiffness lasting two-three hours and heel pain. On physical