Tumoral calcinosis (TC), a calcium hydroxyapatite-based mass, is common in the extremities and hips, but has rarely been reported in the spine, and has never been reported within the spinal cord. It may occur sporadically, in familial form, or as a consequence of disorders that promote soft-tissue calcification. Gross-total resection appears to be curative, but the diagnosis of TC is rarely considered prior to surgery. In this report, the authors describe the management of the first case of intramedullary TC located at the T-5 level in a 20-month-old boy who presented with lower-extremity spasticity. Additionally, salient features of the TC diagnosis, radiological patterns, histological findings, treatment, and outcomes are discussed.