This article summarizes and analyzes the clinical manifestations， diagnosis， differential diagnosis， and treatment of two patients with Kennedy disease （KD）. The two patients had the main clinical manifestations of progressive limb weakness， tongue muscle atrophy， and lingual tremor. Laboratory examination showed a significant increase in creatine kinase； electromyography showed peripheral nerve damage of upper and lower limbs involving the sensory fibers； androgen receptor gene testing showed a CAG repeat number of >38. KD is a type of disease with the main clinical manifestation of lower motor neuron lesions. Genetic testing is the gold standard for its diagnosis， and it needs to be differentiated from amyotrophic lateral sclerosis， diabetic peripheral neuropathy， and chronic inflammatory demyelinating polyneuropathy.