Congenital lethal metaphyseal chondrodysplasia, also known as spondylometaphyseal dysplasia or platyspondylic lethal osteochondrodysplasia (Shiraz type), comprises normal normal intrauterine growth, narrow rib cage, rhizomelic shortness of limbs, slight platyspondyly, and severe metaphyseal dysplasia. Metaphyseal dysplasia predominantly occurs in males and all patients with this disorder die shortly after birth because of subacute myocarditis, pulmonary hemorrhage, cortical necrosis of kidneys, and adrenal.