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Osteonecrosis (avascular necrosis) is a relatively common disorder seen by both rheumatologists and orthopedic surgeons. The vast majority of cases are secondary to trauma. However, for non-traumatic cases, there often remains a diagnostic challenge in defining the cause of bone death. The goal of this article is to review data extensively in the medical literature with respect to the pathogenesis of osteonecrosis, its natural history, and treatment.A review of 524 studies on osteonecrosis was performed, of which 213 were selected and cited.Non-traumatic osteonecrosis has been associated with corticosteroid usage, alcoholism, infections, hyperbaric events, storage disorders, marrow infiltrating diseases, coagulation defects, and some autoimmune diseases. However, a large number of idiopathic cases of osteonecrosis have been described without an obvious etiologic factor. Although corticosteroids can produce osteonecrosis, careful history is always warranted to identify other risk factors. The pathogenesis of non-traumatic osteonecrosis appears to involve vascular compromise, bone and cell death, or defective bone repair as the primary event. Our understanding of the pathogenesis of osteonecrosis is now much better defined and skeletal scintigraphy and magnetic resonance imaging have enhanced diagnosis greatly. Early detection is important because the prognosis depends on the stage and location of the lesion, although the treatment of femoral head osteonecrosis remains primarily a surgical one.A better understanding of the pathophysiology, diagnosis and treatment of osteonecrosis will help the physician determine which patients are at risk for osteonecrosis, facilitating early diagnosis and better treatment options.