Campomelic dysplasia is a rare form of congenital dwarfism caused by mutation in the SRY (sex-determining region Y gene)-box transcription factor 9 (SOX9) gene that encodes for normal cartilage development and sex determination. It is characterized by bowing of the femur and tibia and by 11 pairs of ribs. Cervical spine instability and kyphosis are common and may require surgical correction if spinal cord compression occurs. Meticulous monitoring and management of cervical cord compression and instability with multidisciplinary input from the patient’s orthopedic, neurosurgery, and primary care team is essential. Airway obstruction can occur due to the lack of development of the cartilage rings supporting the tracheobronchial tree, leading to early death in infancy. Cleft palate and Pierre Robin sequence are commonly found. Early tracheostomy may be needed to prevent the historically high death rates in infancy and early childhood. Other co-morbid conditions may include congenital heart defects, ambiguous genitalia, and hearing loss. Familiarity with the associated co-morbid conditions is necessary to conduct a safe and successful anesthetic in these patients.