FBN2 and Restrictive ventilatory defect: Congenital contractural arachnodactyly (CCA), also known as Beals Syndrome, is a rare genetic connective tissue disorder of autosomal-dominant inheritance caused by a mutation of the Fibrillin-2 gene (FBN2) on chromosome 5q23 [1]. Phenotypic characteristics of patients with CCA are exceedingly similar to that of Marfan Syndrome and include multiple flexion contractures, arachnodactyly, severe kyphoscoliosis, crumpled pinnae, and muscular hypoplasia [1,2]. Thoracic cage abnormalities may also be present potentially causing restrictive lung disease [1,2].