Diastrophic dysplasia (DTD) is an osteochondrodysplasia that occurs due to a homozygous mutation. It has an incidence of 1 in 22,000–5,000,0000 live births and results in abnormal skeletal and cartilaginous growth. Individuals with DTD can present with a spectrum of co-morbidities, which can be challenging to anesthetic management. Airway abnormalities such as micrognathia, laryngotracheomalacia, and cervical spine instability may present difficulty with bag mask ventilation and tracheal intubation. Shortened limbs can make for challenging intravenous access and positioning. Restrictive lung disease due to thoracic dystrophy is common. Spinal deformities such as kyphoscoliosis and spinal stenosis make neuraxial anesthesia difficult.