Background: Lingual thyroglossal duct cysts are rare, representing only 0.5% to 3% of all thyroglossal duct cysts cases (TDC). They can present in early infancy with severe respiratory and feeding symptoms, often posing a diagnostic challenge. The aim of this report is to describe a rare case of a lingual TDC in an infant and the successful transoral surgical approach used. Case presentation: A 4-month-old female patient presented with respiratory stridor, wheezing, and difficulty sleeping and breastfeeding since her first week of life. Flexible laryngoscopy identified a nodular lesion at the base of the tongue, while computed tomography confirmed a 1.3 cm hypodense nodular lesion at the foramen cecum, with a normally positioned thyroid gland. The patient underwent transoral resection with drainage of mucous content. Healing occurred by secondary intention, resulting in immediate respiratory improvement and a favorable outcome, with no recurrence over a 2-year follow-up period. Conclusion: This case underscores the importance of early diagnosis using laryngoscopy and imaging to differentiate lingual TGDC from more common pediatric conditions, such as laryngomalacia. The transoral surgical approach proved to be a safe and effective treatment, ensuring long-term resolution of symptoms.