The authors describe a case of a 52-year-old woman in whom tetraplegia developed with neurological respiratory failure due to POEMS syndrome associated with a solitary sacral plasmacytoma. Resection was finally performed after her condition proved resistant to radiation and chemotherapy. The patient showed a dramatic recovery and was ambulatory without tumor recurrence after 5 years and 6 months of follow-up. To the authors' knowledge, there are only 3 reported cases in the literature of bilateral phrenic nerve palsy leading to respiratory failure treated by chemotherapy. This is the first report describing neurological recovery after surgery for pentaplegia due to POEMS syndrome associated with solitary sacral plasmacytoma.