Forty-eight cases of the Vogt-Koyanagi-Harada (VKH) syndrome occurring in patients residing in southern California were reviewed. Thirty-six patients were Hispanic and 12 of other racial groups. Symptoms of meningismus, predominantly headache, were present in 32 (67%) cases, but the other characteristic neurologic symptoms, i.e., tinnitus and dysacusis, were present in only eight (17%) and six (13%) cases, respectively. Dermatologic changes were rare; vitiligo occurred in five (10%) patients, alopecia in six (13%), and poliosis in three (6%). In this patient population, extraocular signs and symptoms of the VKH syndrome, other than headache, were unusual. The ocular manifestations of the VKH syndrome are more constant and include iridocyclitis, vitritis, diffuse swelling of the choroid, serous retinal detachment, and optic disc hyperemia. Procedures that may aid in the diagnosis include lumbar puncture, fluorescein angiography, and standardized echography. The ophthalmologist must be prepared to make this diagnosis and initiate treatment with high-dose systemic steroids based on the typical ocular findings even in the absence of other (extraocular) manifestations of this disease.