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Background and Purpose: Tetra-amelia is a syndrome characterized by absence (complete) for all four members and anomalies. The objective of this report was to describe the case of a patient with tetra-amelia underwent arthrodesis of spine to correct severe scoliosis. Case report: Male patient, 12 years old, 41.1 kg, carrying tetra-amelia, underwent surgery to correct spine with severe scoliosis spine arthrodesis between T4 and L1. Discussion: The management of the patient with tetra-amelia becomes a challenge for the implications of the airway, intraoperative monitoring, fluid replacement and response to vasoactive drugs. This syndrome is uncommon and rare reports in the literature also.