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.