Background: Chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) is a rare, acquired polyneuropathy, especially in children, affecting the peripheral nervous system. It most commonly presents in a symmetric, proximal and distal, sensorimotor fashion. Immunosuppression and immunomanipulation are treatment modalities. This is an update of the 14-year-old male presented, at the 2024 CNSF meeting, with severe progressive CIDP who became refractory to steroid and IVIg but responded to Rituximab. Methods: At the age of 16, at a stage of recovery where he had regained MRC grade 5 strength diffusely, all therapies were sequentially tapered (prednisone & IVIg) or withdrawn (rituximab). He did well for approximately 6 months at which point he relapsed severely to the point of needing a walker. Repeat electrodiagnostics confirmed latency, amplitude, and conduction velocity deteriorations as compared to previous. As a result, prednisone, IVIg, and rituximab were reinstated. Results: As per the first exposure to rituximab, the patient began to respond roughly 3 months after the retreatment with targeted anti-CD20 therapy. He has again made a complete recovery physically but not electrophysiologically. This is the same pattern of response that was originally manifested. Conclusions: Rituximab appears to be an effective treatment for severe IVIg-refractory pediatric CIDP.