Abstract Charcot-Marie-Tooth disease (CMT) is one of the most common hereditary neurological disorders and damages peripheral nerves that results in motor and sensory disturbance. Association of medulloblastoma (MBL) with CMT has been rarely reported. A one-year-old male was referred to our hospital because of cerebellar mass. He had partial resection of the tumor, and was pathologically diagnosed as having desmoplastic nodular medulloblastoma. He received chemotherapy according to the HIT protocol, however, developed severe peripheral neurotoxicity in the initial stage of the treatment. Reinvestigation of family history revealed his mother, grandmother, and aunt had muscle weakness. We suspected he had an inherited neurological disease including CMT, and discontinued administration of vincristine. Fluorescence in situ hybridization analysis detected duplication of PMP22 gene located on 17p11.2, confirming the diagnosis of CMT1A. He completed the rest of chemotherapy without vincristine, and remained in complete remission for four years from the end of treatment. In the literature, there are reports of patients with CMT who developed MBL and were complicated with severe peripheral neurotoxicity due to the use of vincristine. The present case, along with previous reports, suggests that medulloblastoma can develop in patients with CMT and reminds the importance of recalling the possibility of CMT when patients develop severe chemotherapy-induced peripheral neurotoxicity upon use of vincristine. Desmoplastic nodular medulloblastoma may be successfully treated by chemotherapy without vincristine.