The clinical, electromyographic, and histologic characteristics of a 17‐year‐old girl with reducing body myopathy are described. She is, to our knowledge, the oldest reported case and the only patient described with severe mitral valve prolapse and scoliosis. Electromyography demonstrated spontaneous positive sharp waves and fibrillation potentials with many lowamplitude, short, polyphasic motor unit potentials. The right deltoid muscle was chaacterized by focal areas with large fibers associated with increased endomysial connective tissue and “split” fibers. Purple‐gray sarcoplasmic masses stained with trichrome were PAS‐negative, appeared as “empty” spaces with both ATPase and NADH‐TR, and stained darkly with menadione NBT. The features described expand the clinical presentation of this myopathy, and may lead to a better understanding of its etiology.