Perlman syndrome, also known as renal hamartomas or nephroblastomatosis, is a rare hypertrophic disorder associated with an increased risk of bilateral renal tumors at an early age (Astuti et al. 2012). This condition is characterized by neonatal macrosomia, visceromegaly, fetal overgrowth, Wilms tumor, polyhydramnios, nephroblastomatosis, neonatal mortality, and dysmorphic facial features, plus macrocephaly. The characteristic features were first described in 1970 by Liban (Liban and Kozenitzky 1970) in two siblings of a consanguineous Yemenite Jewish family. Perlman et al. later described five affected siblings in 1986 (Perlman et al. 1986). Moreover, Neri et al. (2013) delineated the syndrome in a family with two affected children and 14 additional cases of Perlman syndrome were subsequently documented in literature.