A chordoma is a slow growing, locally invasive, low-grade tumor belonging to the sarcoma family. It mainly affects the sacrum and skull base. We present a case of thoracic chordoma initially presented with epidural hematoma (EDH), which is a rare clinical entity. We reported this case, and also performed a PRISMA-driven systematic review to summary the similar cases in the literature. This review includes the clinical characteristics and outcome of thoracic chordoma. Our case involves a 60-year-old male who, despite no history of trauma, presented with acute paraparesis. An epidural hematoma was identified at T6 level, leading to a surgical intervention involving T4-6 laminectomy and fixation. Sixmonths subsequent to surgery, the patient experienced progressive lower limb weakness and spasticity. Computed tomography (CT) exhibited erosion of T6 and an associated aggressive mass. Magnetic resonance imaging (MRI) revealed a large heterogenous soft tissue mass arising from the vertebral body and right pedicle of D6, protruding in the epidural space and compressing the spinal cord focally at this level. The mass measured approximately 5×4×3.5cm. Magnetic resonance myelography indicated a filling defect at T5–6 level, confirming the intraspinal location of the soft tissue lesion. Complete excision of the mass confirmed the diagnosis of thoracic chordoma. Postoperative follow-up demonstrated notable improvement in the lower limb spasticity and paraparesis, and the patient started adjuvant radiotherapy. This case underscores the importance of maintaining a high index of suspicion when evaluating presentations resembling EDH.