This is a case report of the Roussy-Levy syndrome. The patient is a 22year-old male with with gait ataxia, pes cavus, tremor on both hands, dysequilibrium and peroneal muscular atrophy. On family history, his grandfather, father, aunt and younger brother were affected similar symptoms but slightly milder. Abnormal signs on neurologic examinotion were atropy by of both legs, high arched feet, decreased position and vibration sensation in both lower extremities, absent DTRs in all extremities, positive Romberg test on closed eyes and intention tremor of both hands. On EMG study, there is denervated potentials in tested muscles. On sensory nerve conduction velocity study, no potentials was appeared and on motor NCV, prolonged terminal latency (6.2-8.6 m/sec)and delayed NCV (31-36m/sec) were appeared. His symptoms have been slowly progressed and condition is relatively stable at present.