This report describes a 42-year-old man who presented with an α-type spinal deformity with a Cobb angle of 224.9° and associated spinal cord rotation greater than 90°. Preoperative imaging revealed extensive spinal deformity, and 3D modeling confirmed the α-type nature of his deformity. Intraoperative photography demonstrated spinal cord rotation greater than 90°, which likely contributed to the patient’s poor neurological status. Reports of patients with Cobb angles ≥ 100° are rare, and to the authors’ knowledge, there have been no published cases of adult α-type spinal deformity. Furthermore, very few cases or case series of spinal cord rotation have been published previously, with no single patient having rotation greater than 90° to the authors’ knowledge. Given these two rarities presenting in the same patient, this report can provide important insights into the operative management of this difficult form of spinal deformity.