Eighty-two children (ages 6 months to 12 years) with clinical and/or histopathologic diagnoses of vitiligo were evaluated; 35 were male and 47, female. Fifty-six were black, 25 white, and 3 classified as "other." Children were compared with control groups of children with other skin diseases and with adults with vitiligo. Children had an increased incidence of segmental vitiligo (p less than 0.01). Children had an increased incidence of autoimmune and/or endocrine disease and also of premature graying in their immediate and extended family members (p less than 0.001). Six of 33 children with vitiligo tested had positive organ-specific serum autoantibodies, which was a higher incidence than in the control group of children (p less than 0.05). Eighteen percent of children treated with topical psoralens and long-wave ultraviolet light (PUVA) therapy had an acceptable response, which was less than an adult group similarly treated. We have found childhood vitiligo to be a distinct subset of vitiligo, showing increased segmental presentation; strong autoimmune and/or endocrine disease background and high incidence of premature graying in the families of affected children; the presence of organ-specific serum autoantibodies and a poor response to topical PUVA therapy.