Abstract Objective Kaposi sarcoma (KS) of the larynx is a rare disease with few cases reported in the literature. This study aims to provide a comprehensive review of laryngeal KS, including patient characteristics, treatment, and clinical outcomes. Data Sources PubMed, CINAHL, SCOPUS, and Cochrane Library. Review Methods A systematic review of the published English literature was conducted. An electronic search and bibliographic examination of articles pertaining to laryngeal KS were performed. Demographic data, tumor site, treatment strategies, follow‐up, and outcome were analyzed. Results A total 77 cases from 50 articles were included in the review. The mean age was 47.6 years (range, 8‐81). There was an 8.6:1 male:female ratio. The most common presenting symptoms were dyspnea (n = 35) and hoarseness (n = 25). Laryngeal KS arose most frequently in the supraglottic region (n = 16). Chemotherapy alone (n = 27) was the most common treatment modality in patients with AIDS‐associated KS, and surgical excision alone (n = 7) was most common in patients with other subtypes of KS (eg, classic, transplant associated). Average follow‐up was 20.4 months (range, 0.75‐120). Most patients with AIDS‐associated KS died of other causes (n = 25), but most patients with other subtypes of KS were alive with no evidence of disease at follow‐up (n = 13). Conclusion This review contains the largest pool of laryngeal KS cases to date. Long‐term outcomes were generally unfavorable, often due to advanced HIV disease at the time of diagnosis.