A 55-year-old woman presented with bilateral neuropathic pain of the upper limbs, motor palsy of the right arm, urinary dysfunction, and postural dizziness. MRI showed an intramedullary cervical tumor with a solid portion extended from C1 to C3 surrounded by a cystic portion. A macroscopic complete resection was performed and histological examination confirmed the diagnosis of ependymoma. Postoperatively, the patient's neuropathic pain and postural dizziness worsened, with syncopal attack while upright because of severe orthostatic hypotension (OH). On physical examination, her supine systolic blood pressure was 130 mmHg and fell to 80 mmHg while sitting with no change in heart rate. We found motor palsy of the left arm, bilateral ataxia, and urinary retention. Three months later, the patient was still bedridden, notably because of the OH. After 6 months, with the association of preventative measures of OH and high doses of a direct alpha1-adrenoreceptor agonist, a vasoconstricting agent, the patient recovered an independent gait permitting her to walk unassisted. The main causes of OH include medication, nonneurogenic causes such as cardiac insufficiency, and central or peripheral neurogenic causes such as diabetic insufficiency. Brainstem tumors are known to provide severe OH but this symptom has been seldom described in a purely spinal cord lesion. We report an interesting case of severe OH that had complicated the surgical treatment of a high cervical spinal cord ependymoma and we review the literature.