Hematopoietic stem cell transplant has been used in the treatment of multiple myeloma. Its use in the treatment of POEMS syndrome is still under investigation. We describe the clinical course and long term follow-up of a patient with POEMS syndrome unresponsive to standard chemotherapy. The patient had rapid and dramatic improvement in neuropathy and dermatologic features after autologous stem cell transplantation. A review of the literature is also presented. High-dose chemotherapy followed by stem cell transplantation is a potential treatment option for patients with POEMS syndrome.