The present study aims to investigate the benefits induced by physical activity/practiced sport in Charcot-Marie-Tooth 1A (CMT1A). Patients were divided into sport and no-sport groups according to their sports performance habit. Thirty-one patients were enrolled, of which 14 practiced sports and 17 did not. Clinical assessments were administered to evaluate disability, self-esteem, depression, quality of life, and pain. Statistical analysis revealed significant differences in terms of gender in the no-sport group compared to the sport group ( = 0.04). Regarding the quality of life, physical function ( = 0.001), general health ( = 0.03), social function ( = 0.04), and mental health ( = 0.006) showed better patterns in the sport group than no-sport group. Moreover, neuropathic pain was reduced in the sport group according to the Neuropathic Pain Symptom Inventory ( = 0.001) and ID-PAIN ( = 0.03). The other administered questionnaires showed no significant differences. Our study confirms that CMT1A patients, who practice sports, with a similar severity of disability, may have a better physical quality of life while suffering less neuropathic pain than their peers who do not practice sports. Results recommend the prescription of sport in CMT1A patients.