Alveolar echinococcosis is caused by the larval stage of the fox tapeworm (Echinococcus multilocularis) and is frequently diagnosed as a space occupying lesion in the liver. The growth pattern resembles that of a malignant tumor with infiltration throughout the liver, spreading into neighbouring organs and metastases formation in distant organs. Thus, one of the prevailing differential diagnoses is liver cancer. Guided by the Tumor-Node-Metastasis (TNM) system of liver cancer, the European Network for Concerted Surveillance of Alveolar Echinococcosis and the WHO Informal Working Group on Echinococcosis proposed a clinical classification for alveolar echinococcosis. It was designated as PNM system (P = parasitic mass in the liver, N = involvement of neighbouring organs, and M = metastasis). As for TNM in oncology, single PNM categories were combined into four stages, I to IV. The system was developed by a retrospective analysis of 97 patients' records from two treatment centers (Besançon/France and Ulm/Germany). Recently, this WHO classification was applied to 222 patients in 4 clinical centers around the world (Besançon/France, n = 26; Urumqi/China, n = 46; Sapporo/Japan, n = 58; and Ulm/Germany, n = 92). All patients could be classified who had been diagnosed in the period from January 1998 to June 2005. The stage grouping indicated center differences, but appeared to segregate patients according to various treatment regimens. The WHO classification not only serves as a tool for the international standardization of disease manifestation but also aids to evaluate the outcome of a chosen diagnostic and treatment procedure in different treatment centers in Europe and Asia.