Isolated leukonychia totalis is a rare condition of nails with mainly an autosomal dominant pattern of inheritance. In this report, we present three sibs (a sister and two brothers) with isolated congenital leukonychia totalis, without any manifestation among parents and other family members. The sibs had similar facial features and were offspring of consanguineous Egyptian parents. We discussed possible mechanisms of inheritance and suggested an autosomal recessive mode of transmission. © 2011 Wiley-Liss, Inc.