Charcot neuroarthropathy (CN) is a systemic disease characterized by progressive bone loss and destruction, which is usually closely related to diabetes, HIV, etc. However, CN caused by syringomyelia accounts for only 5% of CN cases; the shoulder and elbow are most often involved, and the hip joint is rarely affected. As a rare factor, cervical spondylotic myelopathy (CSM) can be associated with syringomyelia, which is scarcely reported in the literature. Here, we present the first case report to date of CN of the hip caused by syringomyelia secondary to CSM.We describe a 76-year-old male patient who was diagnosed with CSM due to neck pain and weakness of limbs 16 years ago. Four years ago, he noticed recurrent swelling of the right hip with pain and was diagnosed with degenerative arthritis. Recently, however, his symptoms gradually worsened, and because of progressive pain, destabilization and weakness of the right hip, he was admitted to our hospital. Through systematic physical, radiographic and laboratory examinations, we finally reached a diagnosis: CN of the right hip associated with syringomyelia secondary to CSM. After comprehensive evaluation of the patient's condition, we performed right total hip arthroplasty. During the follow-up, the patient felt well clinically and could walk independently with a knee brace.We suggest a possible etiological association between CSM and syringomyelia, which may reflect a potential pathogenesis of CN. We encourage clinicians to actively carry out a detailed medical history and comprehensive physical and imaging examinations in patients with joint lesions, especially chronic shoulder neck pain, to rule out the possibility of this association, which plays a crucial role in the early diagnosis of CN. Arthroplasty may no longer be an absolute contraindication to surgical treatment of CN. Reasonable selection of the surgical strategy can markedly improve the clinical symptoms and quality of life of patients.©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.