Benign focal amyotrophy (BFA) is a limited form of anterior horn cell disease with variable benign clinical appearance. Clinical symptoms of cold-sensitive BFA have been described but electrodiagnostic workup on these symptoms has not been done. A 28-year-old man suffering from cold-sensitive weakness and coordination disturbance of a hand underwent electrodiagnostic study while the subject was exposed to the cold. According to this study, the BFA with cold sensitivity may be classified as one type of BFA which can be confirmed by electrodiagnostic study.