A 15-year-old girl is described with the sicca syndrome of keratoconjunctivitis and xerostomia. There was no associated rheumatoid arthritis or other connective tissue disorder, but total lipodystrophy had been present since the age of 9 years. Marked hyperreactivity of the humoral immune system and suppression of cell-mediated immunity was shown. The association of sicca syndrome with total lipodystrophy has not previously been reported.