Camptocormia is an unusual condition characterized by a progressive weakness of the extensor muscles of the spine that cause an involuntary truncal flexion. Occasionally, camptocormia can be the clinical manifestation of an underlying myopathy, including inflammatory or metabolic myopathies. We present a case of a 78-year-old female with camptocormia associated with a mitochondrial myopathy. Additionally, we review the clinical characteristics of three similar, previously reported cases.