Desmoid tumors are locally invasive fibrous neoplasms that arise from musculoaponeurotic structures. We report the first case of a desmoid tumor of the larynx complicating pregnancy. At 21 weeks' gestation fiberoptic and indirect otolaryngologic examination of the patient's larynx revealed a submucosal tumor involving the left true vocal cord, ventricle of the larynx, and false vocal cord. Histopathologic examination revealed areas of extensive fibrosis intermixed with degenerated vocal cord skeletal muscle. Despite a subtotal excisional biopsy, growth of the desmoid tumor continued during pregnancy. Endoscopic evaluation 9 weeks post partum revealed complete regression of the tumor.