An 8-year-old boy with mild congenital lymphedema of both legs was noticed to have a conjunctival lesion of the right eye since birth. Topical corticosteroid eyedrops for slight irritation did not alter the appearance of the lesion. On ocular examination, diffuse conjunctival edema in the inferotemporal and inferonasal quadrants and mild injection of the right eye was noted. There was no edema on the left eye. Three extra rows of eyelashes (congenital distichiasis) were found bilaterally on upper and lower eyelids. Related systemic anomalies included mild aortic coarctation, left ventricular hypertrophy, and simian crease on each palm. Conjunctival edema and distichiasis are important ophthalmic features of the congenital lymphedema distichiasis syndrome.