Systemic sclerosis (SS) is a chronic, multisystemic disease, characterized by inflammation associated with fibrosis. Calcinosis is one of the manifestations of this disorder, observed in 10 to 20% of cases. It is usually located on the extensor surface of the phalanges, peri-articular tissue and near the bone prominences. There are only a few cases reported of SS with vertebral column involvement and spinal cord compression by calcinosis causing serious neurological complications. We describe a fatal case of SS who showed tetraplegia secondary to ectopic calcinosis in the cervical medulla and present a review of the literature on the subject.