Background Emery–Dreifuss muscular dystrophy (EDMD) is an uncommon, gradually progressive X‐linked myopathy, and it could result in rigid spinal deformity. Only a few case reports have described surgical treatment of cervical hyperlordosis and thoracolumbar kyphoscoliosis secondary to EDMD. We report a rare case of EDMD to present the surgical strategies of severe cervical hyperlordosis and thoracolumbar kyphoscoliosis. Case presentation The patient was a 22‐year‐old man with EDMD who had severe cervical hyperlordosis and thoracolumbar kyphoscoliosis. A posterior spinal fusion from T9‐S2 was performed to correct the thoracolumbar kyphoscoliosis at the age of 21 years. Six months later, with an anterior C7‐T1 closing wedge bone‐disc‐bone osteotomy and a posterior–anterior–posterior cervicothoracic fusion from C4‐T4, the cervical deformity was corrected, thus achieving a horizontal gaze. During 1.5‐year follow‐up, no loss of correction was observed. Conclusion Cervical posterior–anterior–posterior closing‐wedge osteotomy combined with long fusion at thoracolumbar spine can be a reliable surgical technique to correct severe spine deformity in EDMD. This two‐stage revision surgical strategy can help restore a horizontal gaze on the basis of a balanced trunk. Cervical deformity in such patients should be corrected in the first stage considering its role as a “driver” of the global spine deformity.