The effects of vitamin A deficiency on urolithiasis were investigated in male rats. A vitamin A-deficient diet caused important changes in the composition of the urine of the treated rats when compared with controls. One of the main effects was a decrease in the concentration of urinary glycosaminoglycans and zinc in the rats receiving the vitamin A-deficient diet. Significant differences were also found in plasma vitamin E and in the relation of vit E/vit A between treated and control groups but, in general, with no important differences in vitamin A. Nevertheless, significant differences in kidney content of vitamin A were observed between both groups. On the other hand, lesions of the cuboidal epithelium that covers the papillae in rats treated with the vitamin A-deficient diet were severe when compared with controls. The vitamin A and E plasma levels in urolithiasic humans were also investigated and compared with those found in a control group. No significant differences were observed in plasma vitamin A levels; nevertheless a significant increase in vitamin E and in the vit E/vit A ratio was clearly observed. These results could be related to a possible deficit of vitamin A in kidneys of stone formers, this being one of the diverse factors that can contribute to urolith development. Moreover, the deficit of important urinary crystallization inhibitors normally found in stone-formers, such as pyrophosphate and phytate, can also be related to the presence of low levels of renal vitamin A which prevents the enzymatic degradation of such inhibitors.