Posterior vertebral column resection is a novel surgical approach for the treatment of progressive kyphosis associated with tethered cord syndrome that was first treated with untethering surgery. A patient with tethered cord syndrome associated with kyphosis first underwent untethering surgery, resulting in progressive kyphosis. Posterior vertebral column resection was performed to correct the kyphosis while shortening the spinal column to prevent the spinal cord from stretch injury. Good correction of kyphosis and reduction of tension on the neural elements were achieved without any neurological deficits. In progressive kyphosis associated with tethered cord syndrome, posterior vertebral column resection after untethering surgery represents a safe and efficacious but technically challenging option.