Osteonecrosis (ON) is one of the serious and well recognized complicationscausing severe pain and disability in patients with systemic lupus erythematosus (SLE), and its manifestation and pathogenesis are only partially understood. This review provide an update of the recent progress in etiology, pathogenesis, diagnosis, and treatment of systemic lupus erythematosus related osteonecrosis (SLE-ON). Despite the concomitant use of corticosteroids, alcohol and obesity, the dysregulated immune micro-environment and the complex pathogenesis of SLE synergistically play important roles in the development of ON. Osteonecrosis of femoral head (ONFH) is the most often involved joint in SLE. The current classification and staging system of ONFH is based on imaging techniques, particularly relating to MRI and CT, for the identification and quantification of necrotic lesions. For SLE-ONFH patients, both SLE specific clinical symptoms and ONFH imaging findings should be comprehensively evaluated. Even though advances concerning bone grafting and arthroplasty procedures have resulted in improved clinical outcomes, early pharmacological treatment at the pre-collapse stage may prevent joint collapse and reduce the joint arthroplasty rate, and this needs to be accounted. Although some progress has been made, considerably more research is needed before we fully understand SLE-ONFH. Future treatments of SLE-ONFH may involve genetic or cell-based therapies that target potential biomarkers, and this will lead to effective measures for saving thefunction of hip joint and preventing osteonecrosis.