BACKGROUND: Little information is available on the causes of death among persons with classic Kaposi's sarcoma (CKS). METHODS: We conducted a population-based study in Italy to identify deceased persons with CKS and the underlying causes of death among them, by reviewing multiple-causes-of-death records. Standardised mortality ratios (SMRs) and 95% confidence intervals were calculated to compare the distribution of causes to that among the same-age general population of deceased persons. The geographical distribution was also evaluated. RESULTS: Of the 946 deaths among persons with CKS, 65.9% were attributable to non-neoplastic conditions and 21.9% to malignancies. For 12.2%, no lethal pathology was identified and CKS was considered as the underlying cause. In 90% of these cases, there was visceral/nodal involvement, therapy-related complications, or neoplastic cachexia. Among persons with CKS who died of other causes, an excess for lymphoid malignancies emerged (SMR = 4.40) ( chronic lymphocytic leukaemia (11.03), non-Hodgkin's lymphoma (4.22), Hodgkin's lymphoma (11.80), and multiple myeloma (2.3)), balanced by a deficit for all solid cancers (0.56), with a marked deficit for lung cancer (0.41). We found an excess for respiratory diseases ( chronic obstructive pulmonary disease (1.86)) and genitourinary diseases (chronic renal failure (6.47)). There was marked geographical heterogeneity in the distribution of deaths. CONCLUSIONS: Though referring specifically to Italy, the results are informative for other countries and populations and all cases of CKS in general. British Journal of Cancer ( 2009) 101, 1085-1090. doi:10.1038/sj.bjc.6605265 www.bjcancer.com Published online 25 August 2009 (C) 2009 Cancer Research UK