Background: CIDP is a rare immune-mediated demyelinating neuropathy that has significant phenotypic variability. A unifying immunopathological mechanism remains elusive, likely due to etiological heterogeneity among the variant presentations. This is best exemplified by the identification of nodal/paranodal antibodies, such as neurofascin 155, in a subgroup of CIDP patients who present with a distinct phenotype. Methods: We present the case of a 39-year-old male who presented with a 2-year history of progressive stocking-glove sensory loss and sensory ataxia. Electrodiagnostics confirmed an acquired demyelinating neuropathy, with serum anti-NF155 IgG4. His case was refractory to standard immunomodulatory therapy, including adequate trials of IVIG, steroids, azathioprine, and rituximab. He also had a non-therapeutic trial of PLEX, methotrexate, and tacrolimus. Results: After cessation of all immunomodulatory therapy for 2 years, he had spontaneous remission of his CIDP and near-complete resolution of electrodiagnostic/clinical abnormalities. Conclusions: This case provides insights into the natural history of NF155 “paranodalopathy” and highlights a unique case of supra-refractory CIDP which underwent spontaneous remission with near-complete resolution. Delayed effect from rituximab was posited as a contributor, however, the patient had no clinical or electrophysiological improvement 20-months after initiation of anti-CD20 therapy. Current data suggests the majority of CIDP patients respond to rituximab within 6-12 months.