Superoxide dismutase (SOD) is a crucial antioxidant enzyme involved in the antioxidant pathway during both normal cellular metabolism and different pathologic processes. We investigated the activity of the copper (Cu)-zinc (Zn) SOD1 as well as the level of Cu and Zn in the serum of women with polycystic ovary syndrome (PCOS) and control group. Moreover, the prevalence of rs2070424 gene polymorphism of the enzyme in the course of PCOS was evaluated. Significantly lower activity of SOD 1 and Cu, Zn concentration was found in the group of women with PCOS than without the syndrome. Insulin resistance in the group of women with PCOS caused a further SOD1 activity decrease, while Cu concentration and the value of Cu/Zn was increased when compared to women with normal insulin levels. Furthermore, we assessed for the first time the rs2070424 polymorphism of SOD1 in the women with PCOS, and in these patients we detected dominant variant AA (93.3%). Due to a small number of other genotypes, it is difficult to state if lower SOD1 activity was strictly associated with the AA variant or if other factors play a crucial role, but this should be taken into account.