Choroid plexus carcinomas (CPCs) are rare malignant tumors of neuro-ectodermal origin, accounting for less than 1% of all intracranial tumors. The recurrence rates of CPCs are very high and typically occur in the short-term following surgery, even after gross total removal. Here we present a rare case of CPC with spinal metastasis, which occurred long after its initial presentation. A 25-year-old woman with a history of increased intracranial pressure underwent resection for a tumor of the fourth ventricle, with a histopathological diagnosis of CPC. After tumor resection, she received 30 Gy of radiation therapy to the craniospinal axis and 20 Gy to the primary site, followed by nimustine hydrochloride chemotherapy. The residual lesion completely responded to these treatments. She suffered sensory loss in the sacral region 13 years later, followed by refractory skin ulcer in the sacral region 17 years after the initial treatments. Magnetic resonance imaging at 17 years after the initial treatments showed tumor in the sacral region, which was enlarged upon follow-up after 18 months, causing incontinence and loss of urinary intention. She underwent tumor resection, with a histological diagnosis of recurrent CPC. She received salvage re-irradiation. This case shows that CPC can spread via the cerebrospinal fluid pathways and cause spinal metastasis, with relatively slow clinical course. The present case suggests that patients with CPCs may need long-term follow-up imaging of the total neural axis to identify late recurrence at both the primary site and spinal metastasis.