Charcot-Marie-Tooth disease (CMT) was initially described in 1886. It is characterized by defects in the peripheral nervous system, including sensory and motor neurons. Although more than 80 CMT-causing genes have been identified to date, an effective therapy has not yet been developed for this disease. Since Drosophila does not have axons surrounded by myelin sheaths or Schwann cells, the establishment of a demyelinating CMT model is not appropriate. In this chapter, after overviewing CMT, examples of Drosophila CMT models with axonal neuropathy and other animal CMT models are described.