Lyme disease is a rare tick-borne multisystemic infection caused by Borrelia burgdorferi. Different neurological conditions were reported in the disease. In this article, we present a 15-year-old patient hospitalized with ataxia who was diagnosed with Lyme neuroborreliosis. Intravenous immunoglobulin and ceftriaxone treatment was applied to the patient for 4 weeks. However, ataxia did not recover, upper and lower muscle weakness developed, and deep tendon reflexes diminished during follow-up. The patient was diagnosed with Guillain-Barre syndrome arising from B. burgdorferi. Second dose of intravenous immunoglobulin treatment was started for 5 days but the patient didn't recover. Therefore administration of plasmapheresis was decided. All symptoms relieved following the plasmapheresis. The effect of plasmapheresis in pediatric neuroborreliosis has not been documented before. This study highlights that plasmapheresis could be a useful alternative for pediatric neuroborreliosis cases. J. Clin. Apheresis 31:476-478, 2016. (c) 2015 Wiley Periodicals, Inc.