PURPOSE: This report describes a rare autochthonous case of human D. repens infection in Austria. Dirofilariosis is a mosquito-borne parasitic infection that predominantly affects dogs. Human D. repens infections have primarily been reported in Mediterranean countries, but are emerging throughout Central and Northern Europe. METHODS: The worm was removed surgically and identified using PCR and DNA sequencing. The consensus sequences were compared against reference sequences of Dirofilaria repens from GenBank. RESULTS: The 56-year-old woman acquired the infection, which presented as a subcutaneous nodule, in Vienna, Austria. This is the second autochthonous case of human D. repens infection in Austria. CONCLUSION: The reasons for the emergence of D. repens and other parasitic infections in Central and Northern Europe are manifold, including climate change and globalization. This case demonstrates that with the growing number of D. repens infections, health care professionals must place further emphasis on emerging infectious diseases to ensure appropriate diagnostics and treatment in the future.