Castleman disease was first described more than 60 years ago. It is a rare and heterogenic group of diseases characterised by lymphadenopathy with typical morphological features associated with general inflammatory symptoms and laboratory deviations. The first international diagnostic criteria of this disease were published in 2017 and are detailed in this article. In the USA, the incidence of this diseases is estimated at 5/1 million inhabitants. The incidence in the Czech Republic has not been determined as yet. However, it is possible that some cases are considered to represent a systemic inflammatory response of unknown origin and are thus not diagnosed properly. The aim of this text is to recapitulate the manifestations of this disease and present the first international diagnostic criteria published in 2017.