AbstractChronic inflammatory demyelinating polyradiculoneuropathy (CIDP) affects various components and segments of the peripheral nervous system differently, and thus there can be phenotypic heterogeneity. We report a 47‐year‐old woman with chronic sensory disturbances and proximal weakness limited to the legs. Motor and sensory nerve conduction studies were normal. Somatosensory evoked potentials and imaging indicated a demyelinating process involving the lumbosacral roots. The patient responded favorably to IVIg. Although she did not fulfill the diagnostic criteria for CIDP we believe this patient represents a restricted regional CIDP variant. Muscle Nerve, 2011