Vocal fold paralysis (VFP) is the second most frequent cause of congenital stridor. Although often due to birth trauma, infection, and brainstem abnormalities, most cases are idiopathic. Infrequently, a family history of VFP is elicited, identifying a role for genetic factors in laryngeal function. This study describes a family in which an autosomal dominant form of familial laryngeal abductor paralysis segregates. The typical physical findings, diagnostic and therapeutic considerations, and possible molecular mechanisms of this disorder are discussed in detail.