A 9-year-old girl presented with a slow-growing and painless mass for 7 months in the soft tissue of the sacrococcygeal region. Magnetic resonance imaging revealed a well-circumscribed solid mass located in the subcutaneous soft tissue of the sacrococcygeal area, but not affecting bone structures. The mass was completely removed, and the disorder was diagnosed as myxopapillary ependymoma. In addition, the MYCN gene amplification status of the tumor was evaluated. Extra-axial ependymomas are very rare tumors with a tendency to metastasis, but they are usually regarded as low-grade ependymomas. Long-time surveillance and follow-up are necessary even after complete excision. Besides, we also discuss the diagnosis of primary soft tissue myxopapillary ependymoma.
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