Vitiligo is a disease of cutaneous pigment cells. Recently we reported that one third of patients with vitiligo had destructive lesions in the retinal pigment epithelium and/or the choroid which were visible by ophthalmologic examination. To determine whether abnormalities of the pigment system of the eye and skin are in fact associated, we examined for vitiligo the skin and hair of 107 patients with active uveitis. Thirty-four patients had an infectious or a known cause for their uveitis and none of these had vitiligo. In contrast, six of seventy-three patients (8%) with idiopathic uveitis had vitiligo or early graying of the hair. Seven other patients with active inflammatory diseases of the ocular pigment system were referred for evaluation of white spots on the skin. One of these patients was legally blind (corrected visual acuity less than 20/200 in both eyes) due to chorioretinal scars in the fovea. The finding that vitiligo is more common than expected in patients with uveitis is significant biologically and statistically. This observation supports our hypothesis that the same systemic factors, probably immunologic, are responsible for destroying pigment cells in the skin (vitiligo) and the eye (uveitis).