We report a new case of aneurysmal bone cyst of the larynx occurring in a 22-year-old man. The lesion manifested with progressive breathing discomfort and appeared as a polypoid pedunculated mass attached to the subglottic mucosa. Microscopically, it featured numerous mononuclear and multinucleated giant cells surrounding cavernous spaces filled with blood. Foci of proliferating spindle cells and mature osteoid tissue could be recognized. There was no apparent relationship with the cricoid perichondrium. Clinical follow-up was negative for local recurrence. Based on this report and a review of the literature, we conclude that aneurysmal bone cyst of the larynx is phenotypically comparable to its bone homologue; however, its microscopic recognition may be difficult, especially on small biopsy fragments. Since it can be confused with several lesions, including telangiectatic osteosarcoma, awareness of this rare appearance of aneurysmal bone cyst is important to avoid unnecessary radical surgery.