Abstract: Intracranial interdigital dendritic cell sarcoma（IDCS）is an extreme rare neoplasm. One IDCS patient was admitted at Guangdong 999 Brain Hospital. Current study reviews the clinical imaging and pathological features, immunophenotypes, and differential diagnosis of IDCS based on literatures, in order to improve clinical understanding of the disease. Key Words: Dendritic Cell Sarcoma, Interdigitating；Brain Neoplasms；Meningioma；Diagnostic Errors