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Primary intracranial smooth muscle tumors are rare. Most cases are related to Epstein-Barr virus proliferation in immunocompromised patients such as organ solid recipients. Only a few cases have been reported in pediatric patients. The clinical features are very variable depending mainly on the location and size of the smooth muscle tumor (SMT) and the pathogenesis is poorly understood. We describe two cases of intracranial SMT localized in the temporal lobe and associated with EBV in immunosuppressed children. A review of the literatur