Osteonecrosis of the humeral head is considerably less common than osteonecrosis of the hip. However, as in the hip, the interaction between a genetic predisposition and certain risk factors may lead to increased intraosseous pressure, loss of circulation, and eventual bone death. The most common risk factor remains corticosteroid use, which accounts for most reported cases. Radiographic staging and measurement of lesion size are predictive of disease progression and can be used to determine appropriate intervention. Recent studies have reported the use of various treatment modalities such as pharmacologics, core decompression with small-diameter drilling, arthroscopic-assisted core decompression, and bone grafting. Prospective, randomized studies are needed to determine the efficacy of these joint-preserving procedures. Newer resurfacing techniques have a role in treating articular surface loss. Hemiarthroplasty and total shoulder arthroplasty are recommended for patients with end-stage disease.