Tumoral calcinosis commonly occurs in the articular soft tissues of the extremities but rarely in the spine. The authors performed surgery to treat lumbar tumoral calcinosis in a patient with scleroderma, in whom symptoms of neurological dysfunction had manifested. This 49-year-old woman presented with low-back pain and gait disturbance. Seven years before presentation, scleroderma had been diagnosed, and the patient had received medical treatment ever since. Imaging revealed tumoral calcinosis centered at the bilateral facet joints between L-3 and L-4, marked stenosis of the spinal canal, L-3 spondylolisthesis, and intervertebral instability. Surgery was performed to excise the lesion en bloc. After neural decompression, posterolateral fusion and pedicle screw fixation were undertaken. Symptoms improved after surgery. In this case, the underlying scleroderma that predisposes to calcinosis and facet joint degeneration due to lumbar spondylolisthesis were probably factors leading to the development of tumoral calcinosis in the lumbar spine.