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¹Department of Neurosurgery, Harran University, Şanlıurfa, Turkey
²Department of Neurosurgery, George Washington University, Washington D.C./USA Patients with Klippel-Feil syndrome (KFS) and associated craniocervical junction abnormalities are at high risk for the development of progressive neurologic deficits. A 60-year-old male patient with Klippel-Feil syndrome was admitted to the emergency room with an 8-year history of a progressive spastic quadraparesis which began with numbness in the right lower extremity. Neurologic status of the patient improved significantly following posterior decompression and stabilization. Dramatic resolution of his myelopathy with the initial procedure led to postponing the second procedure. Early postoperative improvement in neurologic function after the initial procedure may be misleading. For this reason, the two-staged ventral and dorsal decompression was suggested for the restoration and preservation of neurologic function. Keywords : Klippel-Feil Syndrome, lipoma, basilar invagination, surgical management