Systemic sclerosis (SSc) is a systemic, immune-mediated disease characterized by abnormal cutaneous and organ-based fibrosis that results in progressive organ dysfunction including the esophagus. We herein report our experience of a patient with SSc who underwent salvage anterior cervical spine surgery that led to late-onset esophageal perforation.A 57-year-old female had progressive cervical kyphosis after laminoplasty for cervical spondylotic myelopathy. We performed anterior cervical discectomy and fusion using a stand-alone cage. Despite prolonged use of a neck collar, migration of the anterior cage was noted three months after surgery. Rapid progression of kyphotic deformity prompted us to perform revision surgery of circumferential cervical correction. However, conventional posterior surgery was precluded due to the extremely poor condition of her neck, including severely sclerotic skin and atrophic muscles. To address this, she underwent a posterior fusion with the closed technique and C4-C5 corpectomy and bone grafting using a low-profile anterior plate. One year after the surgery, CT and routine upper gastrointestinal endoscopy (UGE) showed no evidence of esophagus injury. She remained asymptomatic thereafter. Over three years after her last surgery, however, follow-up CT incidentally revealed an abnormal air leak around the anterior plate. UGE demonstrated large esophagus perforation with the exposed metal plate. As she had already been on parenteral nutrition due to the disease progression of SSc, we decided not to remove the implant.Potential esophagus perforation after anterior cervical spine surgery, even years later, should be considered regardless of the patient’s symptoms, such as chest pain and dysphagia. Spine surgeons need to be cognizant of the fragility of the esophagus, especially in patients with SSc. For patients with SSc, posterior reconstruction alone is recommended as a relatively safe option, even with a suboptimal skin condition.