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Distichiasis is defined as a double row of eyelashes. Inherited forms of distichiasis have been associated with early and late-onset congenital lymphedema. We report on a child with distichiasis and congenital lymphedema in which it is unknown whether the cause of lymphedema was due to a spontaneous mutation or acquired secondary to an intrauterine infection. Acquired forms of distichiasis are reviewed.