The Balkan region is inhabited by hundreds of endemic species of freshwater molluscs but the information on their distribution, as well as that on the distribution of more common and widespread species, is still insufficient; most areas have no complete inventories of their mollusc faunas. The Zrmanja is one of the largest rivers in the Adriatic part of Croatia. Its freshwater molluscs were studied in the last decade, resulting in a substantial body of distributional data. The main objective of this study was to summarise and analyse the recent distributional information on its freshwater molluscs, with special reference to endemic or threatened species. In total 33 freshwater mollusc species were found in the Zrmanja River and its tributaries at 73 sites. The middle section of the Zrmanja River and its largest tributary, the Krupa River, were the richest studied parts. Dalmatinella fluviatilis Radoman, Tanousia zrmanjae (Brusina) and Islamia zermanica Radoman, endemic to the Zrmanja catchment area, were found in the middle and lower sections of the river. Their occurrence is scattered and limited in area; T. zrmanjae is probably on the verge of extinction. The occurrence of the endemic Belgrandiella kusceri (Wagner), Hadziella sketi Bole and Kerkia sp. in springs or underground waters and an abundant population of Unio elongatulus C. Pfeiffer in the Zrmanja and Krupa Rivers is also noteworthy. The area can be regarded as an important part of the Balkan region, one of world’s hotspots of global biodiversity. Although it is protected, there are still anthropogenic factors that are negatively affecting or may affect the mollusc assemblages.