Degenerative mechanisms such as protein accumulation and vacuolar transformation in the skeletal muscle distinguish inclusion body myositis (IBM) from other inflammatory myopathies. IBM is particularly common in patients over the age of 50 years and inevitably leads to progressive muscle weakness and atrophy. Conventional immunotherapies, albeit effective in other forms of myositis, seem to have only a transient or no beneficial effect on disease progression of IBM. So far, no established evidence-based treatment exists and therapy recommendations are based on expert opinion. Recent clinical trials using monoclonal antibodies such as alemtuzumab or etanercept have failed to demonstrate efficacy. Different treatment studies with drugs that aim at degenerative disease mechanisms are planned or ongoing. This review aims to provide an overview of the current treatment options for IBM.