Hereditary motor and sensory neuropathy type 1 (HMSN-1) is an autosomal dominant disorder, which is usually not associated with neoplastic diseases. The disease predisposes to severe vincristine neurotoxicity. We report a 31-year-old women with recurrent Hodgkin's lymphoma and unrecognized HMSN-1 who developed severe motor neuropathy 3 weeks after the first cycle of treatment including 2 mg of vincristine. HMSN is diagnosed in most cases retrospectively, usually suggested by the observation of foot abnormalities or family history. Recognizing early signs of HMSN, such as areflexia and pes cavus deformity, can prevent severe neurotoxicity of polychemotherapy by avoiding vincristine.