Human dirofilariasis is a rare anthropo-zoonotic disease, mainly detected in Southern and Eastern Europe, Asia Minor, Central Asia, and Sri Lanka. An increasing number of autochthonous Dirofilaria spp. infections has been recently reported in the areas previously considered free of the disease, including northern Europe and the Baltic States. A rare autochthonous case of scrotal dirofilariasis detected in Lithuania was described. Here, a 42-year-old male presented with a 1 cm nodule, limited in the scrotum. A nodule excision was performed. On histological examination, a degenerating roundworm with the features of Dirofilaria spp. (multilayered cuticle, well-developed musculature, focally preserved longitudinal ridges) was detected in the abscess cavity. No additional treatment was needed. Twenty-four publications reporting 28 male genitalia dirofilariasis cases in European countries have been identified. Material and Methods: A rare autochthonous case of scrotal dirofilariasis detected in Lithuania was described. In addition, a literature search was performed on the records on human dirofilariasis in male genitalia in Europe. Results: A 42-year-old male presented with a 1 cm nodule, limited in the scrotum. A nodule excision was performed. On histological examination, a degenerating roundworm with the features of Dirofilaria spp (multilayered cuticle, well-developed musculature, focally preserved longitudinal ridges) was detected in the abscess cavity. No additional treatment was needed. Twenty-four publications reporting 28 male genitalia dirofilariasis cases in European countries have been identified. Different genital localizations were involved, including epididymis, scrotum, spermatic cord, testicular tunics, and penis. In all cases, the final diagnosis was achieved by histological examination. D. repens was a prevailing causative agent. A nodule excision was usually a treatment of choice,however, in four cases, a radical orchiectomy was performed, as malignancy was suspected. Conclusions: Due to the benign character of human dirofilariasis, it is considered a low-priority infection. Nonetheless, the awareness of Dirofilaria spp as a possible causative agent of nodules or tumors in male genitalia is noteworthy for differential diagnostics. Each human dirofilariasis case is of interest from an epidemiological perspective.