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Bovine Citrullinaemia (BC) disease is a genetic disorder that causes increased ammonia in the blood circulation. In order not to spread the disease, it is advisable to avoid the spread of Bovine Citrullinaemia (BC) disease-carrying alleles in the dairy cow population. This study aims to identify the distribution of allele carriers of Bovine Citrullinaemia (BC) in dairy cows in the Enrekang regency using PCR- RFLP method. A total of 80 DNA samples originating from the dairy development center in Enrekang (50 heads from Cendana and 30 heads from Angeraja district). DNA samples were amplified by PCR, PCR products were then cut using Ava II restriction enzymes. Identification of BC alleles carriers were calculated based on genotype and allele frequencies. These research found that about 0.6% of Bovine Citrullinaemia recessive allele frequencies in Enrekang and still relatively very low.