Inclusion body myositis (IBM) is a myopathy classified until now within the group of idiopathic inflammatory myopathies (IIM). Nevertheless, its clinical and histological features are specific and different from the other IIM. It is refractory to corticosteroid therapy. Recently, a few cases of IBM with familial transmission have been described, which is the first report in our country; previous reported cases in literature are reviewed. The similarities of some forms of IBM with muscle dystrophies, rather than with inflammatory myopathies are discussed.