Isolated varicocele on the right is a fairly rare disease, often associated with neoplastic processes affecting the spermatic vein system. According to new data, there is no clear relationship between varicocele on the right and neoplasms. Thus, the etiology isjustifiably related to other conditions. Mirror transposition of internal organsis one of them. Situs inversus totalis (SIT) is a rare autosomal recessive congenital anomaly with an incidence of 1 in 10,000 newborns. The etiology of right varicocele in SIT, given the structure of the right spermatic vein that flows into the renal, with the exclusion of other causes, can be considered similar to left varicocele in normal men, including venous valve insufficiency and venous pressure difference. Indications for surgery and the choice of surgical technique also do not differ. The fact of the rarity of published materials on the treatment of varicocele on the right side against the background of SIT prompted us to share this clinical case.