Chordomas are rare neoplasms of low to intermediate grades, which arise from ectopic remnants of notochordal tissue, presenting a slow growth pattern and locally aggressive behavior. Due to their insidious course, the diagnosis is late, requiring immediate therapeutic intervention. The main prognostic factor is total surgical resection with wide margins. This therapeutic objective is only achieved in 40% to 55.6% of the cases, since chordoma tends to present an aggressive behavior, invading adjacent tissues and neurovascular structures. Currently, the main challenge of sacrectomy is to balance a wide resection with the preservation of the neurological function of the patient. Despite cases of successful gross total resection, local recurrence is an inevitable reality, and the overall survival is relatively low. The indication of adjuvant therapies is not well stablished in the literature, since the response to radiotherapy is not satisfactory for these tumors. The aim of the present study is to present a report the case of a patient with sacral chordoma (SC) who underwent partial sacrectomy and to carry out a brief review of the literature on sacrococcygeal chordomas.