Among patients with vacuolar myopathy, one had Pompe disease and five had myopathy with rimmed vacuoles (hereditary inclusion body myopathy): two of these patients had pathogenic mutations in VCP, one in GNE and one in TIA1. The six patients with necrotizing myopathy and one patient with myopathy NOS were diagnosed with necrotizing autoimmune myopathy based on clinical history, response to pharmacological treatment and 3-hydroxy-3-methylglutaryl–coenzyme A reductase or signal recognition particle antibody positivity. Here, VCP is linked to X-linked myopathy with excessive autophagy.