Subglottic stenosis of congenital origin or acquired within the first 12 months of life are challenging in many aspects. Surgical reconstruction is difficult due to the small anatomic dimensions. Tracheostomy is an additional risk factor attributing to mortality and should be avoided, if possible. In this paper, the most important types of subglottic stenosis in the first year of life are discussed. Conservative, endoscopic, and open surgical treatment options are presented and evaluated. Laryngotracheal reconstruction with autologous thyroid cartilage is the treatment of choice for the majority of significant subglottic stenosis cases in this age group. This technique is comparatively less invasive, versatile, and allows all options for open reconstruction using other techniques in case of recurrent stenosis. Subglottic stenosis in early infancy requires expertise and experience in diagnosis and treatment. Considering the limited incidence, these cases should be managed in a referral center.