Spondyloepiphyseal dysplasia (SED), or spondyloepiphyseal dysplasia congenita (SEDc), is an autosomal-dominant skeletal dysplasia. It is a short-trunk skeletal dysplasia affecting the vertebrae and the proximal epiphyses of long bones. The disease has a prevalence of 3.4/1,000,000 and usually presents at birth. Ossification of the bones may be absent or reduced, leading to bony dysplasia and short stature. SEDc is diagnosed based on careful evaluation using history and physical examination, followed by radiological examination and genetic testing. Diagnosis is crucial for this patient population to ensure close monitoring of co-morbid conditions that may need medical or surgical intervention. Infants with SEDc may display ovoid or pear-shaped vertebrae and later develop platyspondyly and irregular end-plates of the long bones in the childhood years. Other co-morbid conditions to watch for include cervical spine stenosis, instability, and spinal cord compression. The pulmonary system may be compromised due to restrictive airway disease and/or weakness of respiratory muscles from cervical spinal cord myelopathy. Upper airway obstruction due to a short and immobile neck is common and may contribute to further pulmonary compromise. The most feared aspect of an SEDc patient’s anesthetic is potential difficulty with airway management. In this chapter, we discuss the phenotype characteristics of a patient with SEDc with special attention to difficult airway management.