Purpose: To report long-term follow-up of a case of paraneoplastic optic neuropathy (PON) successfully treated with combination therapy. Observations: We present a confirmed case of PON from cutaneous melanoma with nearly six years of meticulous follow-up in which vision was preserved and disease remission was successfully induced with combination mycophenolate mofetil, prednisone, and plasmapheresis therapy. Conclusions and importance: Treatment of PON and long-term follow-up are not well described in the literature. In our case of PON, vision was preserved and disease remission was achieved with mycophenolate mofetil, prednisone, and plasmapheresis. Such information may be useful for future cases of PON.