The humeral head is the second most common site of primary avascular necrosis (osteonecrosis) after the femoral head. There may be many underlying factors that may cause disruption of humeral head`s blood supply. The most common etiologies are steroid use and trauma. Radiological imaging is important in determining the stage and extent of the disease and determining the treatment algorithm. The primary goal of treatment is to maintain range of motion and have a painless joint. Management of the treatment is depend on the stage of the disease and degree of the symptoms. While options such as physical therapy modifications, pharmacotherapy, core decompression and arthroscopy are at the forefront in early stage disease, different arthroplasty techniques can be applied in the later stages. Applying the right treatment at the appropriate time is important both in preventing the progression of the disease and increasing patient comfort.