Osteonecrosis of the jaw (ONJ) is a multifactorial disease in patients with primary or metastatic bone malignancy or osteoporosis undergoing systemic antiresorptive therapy, where pathophysiology has not yet been fully determined. The staging of ONJ is based on severity of symptoms and extent of clinical and radiographic findings. Treatment strategies range from conservative local wound care to aggressive resective surgery of all necrotic bone. The first ONJ cases were reported in 2003 and 2004, and although significant progress has been made in our understanding of the disease, much more work needs to be done to completely explain its pathophysiology.