Pelvic pain as the presenting symptom of demyelinating disease is rare. We report on a 49-year-old female patient that initially had symptoms of pain and anesthesia in the perineum. Symptoms later evolved to include both lower and upper extremity weakness and were associated with enhancing spinal cord lesions on MRI. Recognizing that the patient's disease was localized only to the spinal cord led to an eventual serological diagnosis of neuromyelitis optica (Devic's disease), a demyelinating syndrome that is now considered distinct from multiple sclerosis and that primarily affects the spinal cord and optic nerves. Pelvic pain is an unusual first presentation of this illness. Additionally, this case illustrates the challenges of establishing a diagnosis of neuromyelitis optica. Recognizing the distinct clinical features of this rare illness, referring specifically from a spinal cord or ophthalmological etiology, is essential for its rapid diagnosis, and hence for initiation of appropriate therapy.