Overgrowth syndromes such as Beckwith-Wiedemann syndrome, Sotos syndrome, and Weaver syndrome have an increased risk of neoplasia. Two previous cases of neuroblastoma have been reported in children with Weaver syndrome. We present a third description of a patient with Weaver syndrome and neuroblastoma. In a child with phenotypic characteristics consistent with Weaver syndrome, evaluation for neuroblastoma should be considered.