Glomangiomatosis is benign but may manifest as diffusely, locally infiltrating lesions and recur after simple excision. However, conservative treatment should be advocated. The authors report a recent case in which the lesion occurred in the paravertebral area. The patient was a 39-year-old Chinese man who complained of chronic lumbago for 20 years. The clinicopathological features, in conjunction with the immunostaining pattern and ultrastructural features, confirmed the diagnosis. Glomangiomatosis is an extremely rare soft-tissue lesion. To the best of authors' knowledge, only 10 cases have been reported in the English-language literature worldwide, and the current case is the first to represent a lesion arising from the paravertebral area. The authors review the English-language literature in glomangiomatosis.