Spondyloepiphyseal dysplasia congenita (SEDC) is an inherited disorder of bone growth that results in short-trunk dwarfism, skeletal abnormalities, disorders in vision and hearing, atlanto-axial instability, and cleft palate. This important anomaly of the cervical vertebrae carries the risk of tetraplegia during cleft palate operations. In this case report, we discuss a case of spondyloepiphyseal dysplasia congenita, the perioperative and postoperative measures, and the risk of tetraplegia.
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