Therapeutic plasma exchange (TPE) is not frequently used in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) because it usually gives only a short-term benefit. We report on a 16-year-old boy with renal insufficiency undergoing hemodialysis who developed CIDP and underwent TPE with dramatic long-term response to therapy. Nerve ultrasound and MRI findings are also reported. In our patient TPE was chosen because he was already undergoing hemodialysis. Though it is not considered a first-line therapy in pediatric CIDP, TPE may be a good therapeutic choice also in long-term period. J. Clin. Apheresis 30:364–366, 2015. © 2015 Wiley Periodicals, Inc.