The 43rd World Health Assembly approved the 10th Revision of the International Classification of Diseases (ICD-10) in May 1990 and recommended its implementation by January 1, 1993. The ICD-10 uses a new alpha-numeric code, which provides sufficient code-numbers for all new entities, included in ICD-10 and for further amendments in the future. The extended tabular list of ICD-10 contains HIV-disease (AIDS) and permits separate coding of HIV-disease resulting in various infections, neoplastic and other diseases, such as HIV-dementia complex. Extensions and rearrangements have been made in other groups of diseases, for instance in the chapter on diseases of the genitourinary system. ICD-10 now permits a clear distinction between glomerular and renal tubulointerstitial diseases. The different groups of glomerular diseases can be further characterized by a fourth-digit subdivision of the code-number according to the histopathological findings. Thus, ICD-10 reflects recent developments in medial science. The scientific and practical impact of ICD-coding on mortality statistics, however, largely depends on the use of precise diagnosis and their proper arrangement on the death certificate by the physician, certifying the death. This permits the underlying cause of death to be clearly identified by the coder. The role of the pathologist in this process is stressed. Exact and internationally unified formulation of the diagnosis will be supported in the future by the on-going project developing an International Nomenclature of Diseases (IND). A few volumes of the IND have already been published in English, others are in preparation.