Inclusion body myositis (IBM) is an inflammatory and degenerative autoimmune disease that targets specific muscle groups, causing severe muscle weakness. Exercise training is often contraindicated in myopathies as it may aggravate muscle damage and inflammation. Although some reported positive outcomes in muscle strength of early diagnosed IBM patients undergoing resistance training, there remains uncertainty as to whether exercise could be beneficial and safe in advanced stage IBM. Thus the aims of this research were to evaluate the safety and response of 16-weeks supervised resistance training on the health and muscle performance of an elderly participant diagnosed with advanced stage IBM. It was shown that the training had no adverse effects on the health of the patient. Muscle strength measured at eight weeks and on completion of the intervention, remained the same as at baseline. In conclusion, the exercise programme was found to be safe and seemed to maintain muscle strength in a patient with advanced stage IBM.