The superfamily of ATP-binding cassette (ABC) transporters is a large family of proteins with a wide substrate repertoire and range of functions. The main role of these proteins is in the transportation of different molecules across biological membranes. Due to the broad range of substrates, ABC transporters can transport not only natural metabolites but also various xenobiotics, including antifungal compounds, which makes some ABC transporters key players in antifungal resistance. Alternatively, ABC proteins without transport function seem to be essential for fungal cell viability. In this work, we review the individual subfamilies of ABC transporters in filamentous fungi regarding physiological substrates, clinical and agricultural significance. Subfamilies are defined using well-studied transporters in yeast, which may help to clarify their role in filamentous fungi.