The aim of this study was to determine the incidence and survival of Extramammary Paget’s disease (EMPD) and to describe the possible increased risk of tumours after EMPD. All invasive cases diagnosed between 1990 and 2002 were selected from the RARECARE database. Incidence was expressed in European standardized rates. Relative survival was calculated for the period 1995–1999, with a follow-up until 31st December 2003. Standardized incidence ratios of second primary tumours were calculated to reveal possible increased risk after EMPD. European age standardized Incidence of EMPD within Europe is 0.6 per 1000,000 person years. Five-year relative survival for invasive EMPD was 91.2% (95%CI; 83.5–95.4), 8.6 percent of the EMPD patients developed other malignancies. The highest increased risk of developing a second primary tumour was found in the first year of follow-up (SIR:2.0 95%CI; 1.3–2.9), living in the South European region (SIR:2.3 95%CI; 1.5–3.5) or being female (SIR:1.5 95%CI; 1.1–1.9). Female genital organs displayed greatest increased risk of developing a second primary tumour after EMPD (SIR:15,1 95%CI; 0.38–84.23). Due to the increased risk of a second primary tumour after EMPD a thorough search for other tumours during their follow-up is recommended.