There are four widely recognized subtypes of Kaposi sarcoma (KS): Classic KS, Endemic KS, AIDS-related KS, and Iatrogenic KS. Known risk factors for KS include Mediterranean, Eastern European, or Ashkenazi Jewish origin, residence in Sub-Saharan Africa, as well as immunosuppression, increased age, and male sex. Infection with Kaposi Sarcoma Herpes Virus (KSHV), also known as human herpesvirus-8 (HHV-8), was found to be the etiology of KS in 1994. The virus establishes a lifelong latent infection in the host and undergoes sporadic reactivation of the lytic cycle. Patients with KS can present skin and mucosal lesions, and internal organ involvement. KS lesions typically appear purple or maroon in color and commonly occur on the lower extremities, genital region, and oral mucosa. In advanced disease with KS, lymph nodes, lungs, liver, or the gastrointestinal tract are involved. Diagnosis requires a biopsy of the lesions with histopathological confirmation to avoid misdiagnosis with similar-appearing bacillary angiomatosis. There is no known cure for KS; however, restoring immune function in patients with AIDS-related KS and iatrogenic KS result in regression of both cutaneous and mucosal lesions. Chemotherapeutic agents such as doxorubicin, daunorubicin, and paclitaxel are effective for widespread KS.