Summary The incidence of a malignant disease reflects the genetic and cumulative exposure to the environment of a population. Therefore, evaluation of the incidence and trends of a disease in different populations may provide insights into its aetiology and pathogenesis. To evaluate the incidence of haematological malignancies according to specific subtypes, we used population‐based registry data in J apan ( N = 125 148) and the U nited S tates ( US ; N = 172 925) from 1993 to 2008. The age‐adjusted incidence of haematological malignancies in J apan was approximately one‐half that in the US but has been increasing significantly, whereas no significant change was seen in the US [annual percent change (95% C confidence interval): J apan, +2·4% (1·7, 3·1); US , +0·1% (−0·1, 0·2)]. Hodgkin lymphoma ( HL ) and non‐ H odgkin lymphoma ( NHL ) showed the largest differences in incidence, with the most remarkable differences observed for chronic lymphocytic leukaemia, HL ‐nodular sclerosis, mycosis fungoides and cutaneous T ‐cell lymphoma. HL and NHL are increasing substantially in J apan but not in the US , suggesting that environmental exposures, such as W esternization of the life style may be causing this increase. Differences in the incidence and trends for specific subtypes also showed a marked contrast across subtypes, which, in turn, may provide significant new insights into disease aetiology in the future.