A case of a previously undescribed anomaly of the larynx is presented and offers insight and support of current concepts of fetal laryngeal development. The patient possessed unilateral absence of true and false vocal cords, laryngeal ventricle, and saccule. Current concepts of embryological development would place the development of this anomaly and most of the patient's other multiple anomalies in the period of the 6th to 9th weeks of fetal life. This report lends substantiation to the stages of laryngeal development by demonstrating an aberration in the normal sequential development. Additionally, the time period of this maldevelopment is suggested by the concurrence of multiple other anomalies. Aspects of laryngeal embryology relevant to laryngeal maldevelopment are reviewed.