Abstract Nine horses from ages 5 to 21 years were diagnosed with cutaneous equine sarcoidosis (ES) over an 18-year period. In addition to skin, the lungs were frequently involved, with other organ systems affected less commonly. A predisposition for thoroughbreds and geldings was noted. Cutaneous lesions and signs included crusts, scales, alopecia and pruritus. These were found at various sites, particularly the legs/thighs/elbows, thorax, neck, face and ventral abdomen. Three horses were euthanized shortly after hospitalization; others survived as long as 12 years. Histopathologic stains, immunohistochemistry and polymerase chain reaction assays on paraffin-embedded cutaneous specimens from eight horses for Mycobacterium spp., Coccidioides immitis, Cryptococcus neoformans, Corynebacterium pseudotuberculosis, and Borrelia burgdorferi were all negative. The aetiology of ES is unlikely microbial and continues to be a diagnosis of exclusion. ES, when limited to the skin, is associated with a good prognosis, with either partial or complete response to glucocorticoid therapy in all the surviving horses.