Distichiasis is a challenging condition that may require multiple surgical interventions. Besides ophthalmologic concerns in children, distichiasis may be part of the lymphedema-distichiasis syndrome, which presents with lymphedema of variable time of onset. Other significant systemic disorders such as coarctation of the aorta and varicose veins have been reported in association with this syndrome and must be reviewed for proper patient care. The authors report the case of a 22-year-old male patient who had been treated for distichiasis and followed for 16 years. At his initial evaluation, at 6 years of age, he presented solely with ocular complaints due to distichiasis. Only after 13 years of repeated ophthalmic treatments and continuous follow up, lymphedema was observed. Lymphedema-distichiasis syndrome diagnosis must be considered in children with distichiasis, even in the absence of lymphedema.