A case of centronuclear myopathy is presented, showing a rapid course in a 47-year-old male. Clinical and muscular findings were consistent with the diagnosis of centronuclear myopathy. On nerve biopsy, there was evidence of a regenerative process. Electron microscopy showed proliferation of Schwann cells with numerous Büngner's bands and other anomalies. This rare case with peripheral nerve changes may provide evidence to support the hypothesis of peripheral nerve involvement in centronuclear myopathy.