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Department of Neurosurgery, Şişli Etfal Hospital, Istanbul, Turkey Larsen syndrome is characterized by flat face with hypertelorism, multiple and bilateral congenital dislocations and juxta-calcaneal accessory bone or bifid calcaneus due to a generalized mesenchymal defect involving connective tissue. This rare syndrome sometimes causes severe cervical spinal kyphosis in childhood. In this report a six-year-old female patient with severe cervicothoracic kyphosis due to Larsen syndrome is presented. The patient was operated on through the posterior approach and cervical and upper thoracic pedicle screws were used for correction of the deformity. Larsen syndrome and surgical correction techniques for the severe cervical kyphotic deformities are discussed with the relevant literature. Keywords : Cervical kyphosis, Larsen syndrome, Spinal deformity correction