Autologous peripheral hematopoietic stem cell transplantation (APHSCT) was performed to treat a patient with neuromyelitis optica. We observed that the patient achieved clinical remission after APHSCT during 12 months of follow-up. The patient improved on the expanded disability status scale neurologic assessment and the Scripps neurologic rating scale worksheet scores on follow-up examination compared with baseline. The MRI abnormalities on T1WI and T2WI improved. The postpartum maternal blood helper T (CD3+CD4+ T cell) and the CD4+/CD8+ ratio were decreased, and the cytotoxic T (CD3+CD8+ T) cell increased. This shows that APHSCT in this patient with neuromyelitis optica appears to reduce the frequency of attacks with improvement in disability. However, a larger study with more patients and a longer follow-up would be required to assess the encouraging preliminary results.