We report a patient with craniovertebral anomaly leading to cervical cord compression who presented with disabling postural hypotension. A 60-year-old electrician presented with progressive weakness of the upper and lower limbs, which had started 7 years previously. He had difficulty in holding urine for the previous year and had blacked out on standing for the past 3 months. He had upper limb wasting and lower limb spasticity, with impaired joint position sense. Autonomic dysfunctions included postural hypotension, absence of sinus arrhythmia, impaired Valsalva ratio, and lack of increase in blood pressure on cold immersion and isometric contraction. Cervical spine radiograph and magnetic resonance imaging revealed atlantoaxial dislocation, Klippel-Feil syndrome and osteophytes, resulting in cord compression at C2-C4. Partial and selective damage to the descending autonomic fibres may be responsible for postural hypotension in this patient.