A 4 yr old intact male rat terrier presented with severe bilateral nonresponsive panuveitis. Bilateral uveitis, blepharospasm, conjunctival hyperemia, diffuse corneal edema, peripheral bullous retinal detachment, and secondary ocular hypertension were noted. Ocular lesions progressed despite aggressive medical treatment and were followed by cutaneous depigmentation and crusting along the nasal planum. Intensive oral and topical anti-inflammatory and topical antiglaucoma medications were administered, but the ocular disease progressed. A bilateral enucleation was performed. Uveodermatologic syndrome was diagnosed from histopathologic examination of a skin biopsy as well as histopathology of both globes after bilateral enucleation. To maintain control of the dermatologic lesions, oral azathioprine was initiated, but it was not well tolerated by this patient. Immunosuppressive doses of oral cyclosporine and anti-inflammatory doses of oral prednisone were used to control the depigmentation and crusting skin lesions.