BACKGROUND Hepatocellular carcinoma (HCC) occurring in "noncirrhotic" hepatitis C virus (HCV)-infected patients has been reported; but the exact prevalence or incidence has not been described before. METHODS We conducted a systematic review of literature: Ovid was used to search the literature from January 1, 1990, to September 1, 2008. Articles containing "HCC" keywords (hepatocellular carcinoma, hepatoma, liver cancer) were combined with the word "cirrhosis" or "fibrosis" and with "absence" keywords [noncirrhotic, absence, without]. Two hundred articles were selected and screened according to predesigned exclusion and inclusion criteria. RESULTS Nineteen articles met the inclusion criteria. The estimated prevalence of noncirrhotic HCC ranged from 6.7% to 50.1%. The pooled prevalence estimates for HCV in noncirrhotic HCC ranged from 0% and 68.4% according to the geographic location. Reports from Japan had the highest estimated pooled prevalence of HCV (55.01%) followed by Italy (29.95%). CONCLUSIONS HCV can occur in patients with HCC without cirrhosis, but the true incidence and prevalence are very difficult to ascertain. Further studies are needed to define this group of patients.