In this paper, we report on a family with primary, congenital lymphedema where no variants were found in VEGFR3. As the proband presented with a congenital form of lymphedema resembling that reported by Gordon et al. [6], DNA from the proband was subjected to a VEGFC screen and a splice-site variant (c.361+5G>A) was identified. Here, VEGFC is linked to lymphedema.