An uncommon congenital anomaly of the developing aerodigestive tract is the congenital laryngeal cleft and its extension, the congenital laryngotracheoesophageal (LTE) cleft. Because of the subtle findings sometimes seen with clefts confined to the larynx, the diagnosis may be difficult. The potential problems of airway obstruction and repeated aspiration frequently associated with laryngeal and LTE clefts mandate early surgical repair. Over the past 3 years, 14 patients with congenital laryngeal or LTE clefts have been managed at Children's Hospital Medical Center, Cincinnati, Ohio. This report details our experience and philosophy regarding the diagnosis and management of the condition, and presents the histopathologic findings of a case of complete LTE cleft in which surgical repair was delayed and death resulted.