Background: A rare angioproliferative condition of the larynx, Kaposhi sarcoma typically affects the skin. Immunosuppressive treatment following organ transplantation and human immunodeficiency virus infection are the causes. Every type of Kaposi sarcoma has human herpesvirus-8. Laryngeal kaposi sarcoma is uncommon in immunocompromised patients; since its initial identification in 1965, 18 cases have been documented globally. A CO2 laser-assisted laryngeal microsurgery is performed through the mouth cavity to remove tumor when kaposi sarcoma of the larynx obstructs the airway. Case report: A 77-year-old woman complained of hoarseness, dry mouth, odynophagia, and dysphagia three months prior when she arrived at the Mongolian-Japan Hospital. Two years ago, she acquired hard, sensitive lumps that were palpable on her right arm, left ankle, and right thigh. At that time, she was diagnosed with Kaposiform hemangiodermatitis and treated at the National Center for Dermatology. HIV test results were negative. Immunohistochemistry: CD31 +/-, CD34 /+/. Using flexible nasopharyngeal endoscopy to get the diagnosis: There was a mass that was about 1.5–2–5 cm in diameter, bluish in color, smooth and movable, and spongy and vascular on the larynx, on the nasopharynx, behind the palatine tonsills, and supraglottic. Surgery: Through the use of Kleinsasser laryngoscopy and a 0-degree endoscope, pathological tissues were extracted under general anesthesia using a laryngeal microsurgical instrument and a laparoscopic bipolar coagulator. The tissues were then sent for histological evaluation, which revealed Kaposi sarcoma, sarcoma grade 1. Results of treatment Pain decreased and quality of life increased following surgery. Upon nasopharyngeal endoscopy, the vocal cord mobility was normal and the surgical incision was clean. Conclusion: Kaposi sarcoma is an extremely uncommon illness. A lower quality of life and further issues can be avoided with an early diagnosis and suitable therapy. It also needs to be continuously monitored because it is a potentially repeatable disease.