In this paper, we have summarized the current knowledge of earthworm fauna in North Macedonia. This list presents earthworm diversity, general ecology, distribution, and zoogeographical types for the first time. The complete list of earthworm taxa of North Macedonia comprises 59 species and subspecies, of which 57 taxa belong to the family Lumbricidae and one taxon each of the families Acanthodrilidae and Criodrilidae. Dendrobaena is the most speciose genus (18 taxa). The family Lumbricidae of North Macedonia is characterized by a large number of narrow range Balkanic endemics (9 taxa = 15.3%), together with the occurrence of broad range Balkanic endemic (7 taxa = 11.8%) and Vardar endemic species (5 taxa = 8.47%). A high representation of endemic species (35.6%) indicates a high degree of autochthonous earthworm fauna.