This study aimed to document and describe a case of a laryngeal pilar cyst and to review the literature.We describe the case of a 65-year-old woman with a laryngeal pilar cyst presenting with occasional ear pain and positional dyspnea, with imaging studies suggesting external/internal laryngocele. We also review the existing clinical literature.Pilar cysts are adnexal skin lesions most commonly found in the scalp of elderly women. They generally have a benign course, but in rare instances single or multiple foci of proliferating cells can lead to the neoplastic formation of proliferating trichilemmal cysts, which carry malignant potential. Depending on the location of the cyst, pilar cysts may also present functional challenges for the patient.Herein, we describe a pilar cyst in and around the larynx appearing initially as a laryngocele. Pilar cysts may present surrounding the larynx and may be mistaken for a vast array of pathologies. It is important to keep the differential broad when evaluating laryngeal masses.