Kaposi's sarcoma (KS) is the rarest cancer among Iranian. This study was conducted to define the epidemiology of KS in Iran. The data of National Cancer Registry (NCR) reports from 2003-2006, Tehran Population Based Cancer Registry (TCR) from 1998 to 2001, and a literature review of the published articles until Aug. 2007 was conducted, the search strategy included all papers containing "Kaposi"were combined with the word "Iran" in their titles or abstracts. In males, the annual age-standardized incidence rate was from 0.10 to 0.17 per 100,000 and in females was from 0.06-0.08 per 100.000. Peak incidence was at ages 50-79. It is more common in male than female. The Male/Female ratio has different reports from 3.2/1 to 1.8/1. Although, there were no published reports about HHV-8 or HIV/AIDS associated with KS in Iran, the elderly might be a common associated factor for KS. The prognosis of KS after renal transplantation is good; simple reduction or cessation of immunosuppression may result in partial or complete remission of the disease.