The Alto Ribeira karst area, southeastern Brazil, is a high-diversity area for troglobites. Three species of freshwater gastropods Potamolithus occur in the area: P. ribeirensis, only found in epigean waters at the Iporanga and Ribeira rivers; P. troglobius, which is endemic to the Areias cave system; and P. karsticus, a troglophilic species from Calcário Branco Cave and an epigean stream nearby. We investigated their distribution based on shell morphology and internal anatomy of epigean species, troglophilic populations, and troglobitic species. Distribution patterns of Potamolithus were compared to those of other aquatic taxa from the region (such as crustaceans and fishes). Besides the three species already described for the region, we recorded 12 additional ones, for a total of 15 species/morphs (six troglobites, seven troglophiles, and two epigean). Potamolithus spp. are restricted to micro-basins and/or caves, showing small areas of distribution and probably a high degree of endemism. Geomorphology (irregular landscape, with limestone outcrops intercalated with insoluble rocks, which probably act as geographic barriers for cave populations), paleoclimatic evidence, and ecological/biological factors, such as the low degree of mobility of these gastropods (sedentary habit), explain the distributional patterns. We observed troglomorphisms such as reduction/absence of eyes and pigmentation (body and periostracum), and a coiled intestine. Apparently, there is no cause-and-effect between miniaturization and intestine coiling for Potamolithus, in contrast to observations for other cave snails. Potamolithus snails are threatened in the region due to water pollution, uncontrolled tourism, and overcollection.