In our study, we estimated the frequency of haplotype for cholesterol deficiency (HCD) carriers in the Russian Holstein cattle population. We studied 1817 random samples of cows born in 2010-2017 from ten herds and 331 cows and heifers from the other three herds born in 2016-2019, fathers or fathers of mothers of which were HCD carriers. The method of AS-PCR was used for animals genotyping. In the first group of animals, the incidence of HCD carriers was 8.09%, and in the second one - 23.26%. Our results demonstrated the necessity to test cows for the carriage of the HCD genetic defect in the Russian population of Holstein cattle.