Background Motor neurone disease (MND) is a chronic, progressive and currently incurable neurodegenerative disorder. Although pain as a symptom appears in many patients with MND, it is often misdiagnosed as other diseases when occurs before the onset of weakness. Patients are often assigned to non-neurological departments due to the atypical symptoms, which can lead to diagnostic delay and inappropriate treatment. Objective To analyze the causes of misdiagnosis and improve the clinician's understanding of neck pain in patients with MND. Methods We reviewed relevant literature and retrospectively reported a misdiagnosis case of MND-associated neck pain. Results A case of MND presenting prominently as neck pain was suspected of suffering from cervical spondylosis and wrongly assigned to orthopedic clinic. When eventually being diagnosed as MND, his neck pain was found to be caused by intracranial hypertension (ICH) resulting from hypoxia via insidious respiratory failure through ventilator insufficiency. Conclusion Careful evaluation of the clinical progression of the symptoms, extensive EMG and nerve conduction study, as well as the establishment of better clinical approach to the diagnosis and higher public awareness allow a reduction of misdiagnosis.