This report describes the unusual association of bilateral abductor vocal cord paralysis (BAbVCP) and Charcot-Marie-Tooth disease in a boy and his natural mother who have been followed for eight years. The boy initially presented with life-threatening respiratory distress at age ten years; BAbVCP was documented by direct laryngoscopy. Mirror laryngoscopy confirmed BAbVCP in the mother. Neurological diagnosis was made by history, physical examination, electromyography, and nerve conduction velocity studies. The BAbVCP may represent an additional genetic marker within the spectrum of heredodegenerative disorders. Of clinical importance is examination of voice and respiratory symptomatology of patients with heredodegenerative diseases and neurological work-up of patients with familial vocal cord paralysis. Further genetic and clinical studies of X cranial nerve involvement in heredodegenerative disorders are warranted.