The altitudinal distribution of microfungi associated with Betula ermanii Cham. leaf litter was investigated on Mt. Rishiri (1719 m), northern Japan. Fallen leaves were collected at five sites of different altitudes (approximately 300, 600, 900, 1200, and 1500 m above sea level) along a transect on the northern slope of Mt. Rishiri. A total of 35 species were isolated from Betula leaf litter of which 12 occurred frequently and were regarded as dominant microfungi. Species richness, diversity, and equitability of microfungal assemblages were lower at Site 1500 than at the other sites. Similarities in species composition were relatively high among Sites 300, 600, and 900, but similarities between these sites and sites at the higher altitudes (i.e., Sites 1200 and 1500) were relatively low. Similarity of species composition was relatively low between Sites 1200 and 1500. Cluster analysis showed that the 12 major microfungal species were classified into four groups according to their pattern of altitudinal distribution that peaked at Sites 600, 900, 1200, and 1500, respectively. These results suggest that the diversity and species composition of Betula microfungi are sensitive to environmental changes along the altitudinal transect on Mt. Rishiri, and this is especially so at the altitudes higher than approximately 1200 m. This implies that future environmental changes will have impacts on mountainous ecosystems through the changes in diversity and functional aspects of microfungal assemblages.