Mast cells have become a recent concern in the nephrological world. The development of antibodies to mast cell-specific enzymes, tryptase and chymase, has facilitated the study of mast cells in the kidney. Now, they are investigated immunohistochemically as well as histochemically. There are three types of human mast cells, MC(T), which contains exclusively tryptase, MC(TC), which has both tryptase and chymase, and MC(C), which contains only chymase. Many immunohistochemical studies involving mast cells have been conducted through the use of renal biopsy specimens. As a result, human renal diseases including various glomerulonephritis and pyelonephritis are found to have increased the number of mast cells in the renal cortex, especially in the area of fibrosis. The relationship between the number of mast cells and the prognosis of renal diseases has been proved to be significantly correlated in many reports. The subtypes of mast cells in these diseases are variably present, and no tendency of subtype specificity has been found. With the use of electron microscopically, mast cells are observed to be in contact with other interstitial cells or to infiltrate into tubules. Functionally, human renal mast cells resemble lung mast cells. Inhibitory substances for mast cell proliferation have been found in the mouse kidney. Compared with the results of human studies, mast cells are not found in the interstitum in animal models of renal diseases, except in a few transgenic mouse models and magnesium-deficient rats. Little is known about the exact roles that mast cells play in the renal interstitium. Future studies will hopefully make their characteristics clear.