Congenital subglottic stenosis is rare and as a consequence may not be considered in children experiencing respiratory difficulty at birth. Diagnosis after a child already is intubated complicates the recognition and blurs the boundary between congenital and acquired lesions. This article discusses the anatomy of the larynx, its common anatomic variations, and its response to trauma, a thorough understanding of which is required for the accurate diagnosis and treatment of this complicated problem. The authors discuss evaluation and assessment options to guide treatment.