Kniest dysplasia is a rare genetic disorder, first described in 1952 by Wilhelm Kniest, that is caused by a type II collagen COL2A1 gene mutation. The inheritance is autosomal dominant with variable presentation. We review hallmark phenotype features, including short stature with constricted chest, kyphoscoliosis, progressive stiffness, and enlargement of various extremity joints, myopia, and hearing loss. We explain what causes the key radiographic findings, such as “Swiss cheese” appearance of cartilages and “dumbbell” shape of long bones. We discuss various comorbid conditions associated with Kniest dysplasia. Next, we concentrate on anesthetic management of patients with Kniest dysplasia with difficult tracheal intubation compounded by fear of spinal cord injury. Airway management requires skilled and experienced providers, appropriate planning, and an interdisciplinary approach. We further describe likely challenges, such as intravenous access, positioning problems, and loss of evoked potentials. Finally, we discuss pitfalls for postoperative care, including post-extubation airway injury due to tracheal abnormalities and connective tissue vulnerability as well as the need for close monitoring and respiratory support. We close this chapter with a case scenario that describes a patient seen at our hospital.