The larynx and the remaining components of the upper aerodigestive tract collectively play an important role in undertaking respiration, phonation, and deglutition. Therefore, a variety of pathologies can present with similar symptoms. Systemic diseases, such as rheumatoid arthritis and relapsing polychondritis, may also manifest with laryngeal symptoms and findings, whereas rare pathologies may present with vague presentations. Such scenarios may be attributed to the consequent medical confusion and dilemma in reaching an accurate diagnosis. In this case report, an 11-year-old male presented with airway compromise symptoms, which were later identified and diagnosed as laryngeal embryonal rhabdomyosarcoma.