Abstract
Desmoplastic small round cell tumors (DSRCTs) are highly malignant tumors, with distinct reciprocal chromosome translocation (11;22)(p13;q12). Intracranial metastasis is a very rare complication of this tumor, with only a few cases reported in the literature. To our knowledge, this is the only case presenting an extracranial extension of intracranial metastasis of DSRCT. A 33-year-old man was diagnosed with DSRCT in the pelvic cavity. He presented with a scalp lump and right-sided weakness. A biopsy showed metastasis from DSRCT. Metastatic DSRCT to the brain is extremely rare. Surgical resection followed by adjuvant treatment, including chemotherapy and radiation, is indicated as it has a poor prognosis. Moreover, aggressive treatment is warranted to prevent progression and relapse.