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Benign monomelic amyotrophy is a relatively static form of juvenile onset motor neuron disease, which usually affects unilateral distal upper extremity. In this report, we present an 11-year-old girl, who complaining of muscular atrophy and weakness in right hand for 8 months. Motor nerve conduction studies demonstrated abnormal findings on compound muscle action potential of the median and ulnar nerves of the affected hand, and needle electromyography showed significant abnormal spontaneous activity and neuropathic motor unit action potentials in not only weak and atrophic hand muscles but also asymptomatic contra-lateral hand muscle. Cervical magnetic resonance imaging (MRI) in neutral and flexed cervical positions revealed loss of lordosis. The results of clinical, electrophysiological, radiological findings led to the diagnosis of benign monomelic amyotrophy. Occupational rehabilitation program was conducted with remedial and adaptive approaches, and the positive effects of rehabilitation were shown after 3months.