CFIA said in a statement that the mad cow disease was found in a six-year-old beef cow in Alberta, according to the Canadian Press.
It added that no part of the animal entered the human food or animal feed system and the new case should not affect exports of Canadian cattle or beef.
The agency is searching for the animal's herd mates at the time of the steer's birth and the path of infection. CFIA did not release the birth place of the cow.
Bloomberg reported that the sick animal was born years after Canada and the United States discontinued the use of cattle feed that contained ground-up cattle tissue, the presumed cause of the mad cow disease outbreak.
Mad cow disease has been linked to more than 150 human fatalities across the globe. People contract the disease by consuming meat from infected animals.
Since May 2003, a total of 14 cases of mad cow disease have been reported in Canada.
