Severe combined immunodeficiency disease (SCID) of horses is an autosomal, recessive hereditary disease occurring among Arabian horses. The genetic defect responsible for this disease was recently identified as a 5-basepair deletion in the gene encoding DNA-protein kinase catalytic subunit (DNA-PKcs). Horses with one copy of the gene appear normal, while horses with two copies of the gene manifest the disease. The present report describes a PCR-based test for detection of the gene defect and the results from testing 250 randomly selected Arabian horses. The frequency of SCID gene carriers was 8.4% (21/250). Based on the gene frequency reported here, the authors would expect 0.18% (1 out of 567) of Arabian foals to be affected with SCID based on a random breeding population.