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To investigate the clinical features and radiologic findings in misdiagnosed intracranial infection.A series of 12 uncommon patients with misdiagnosed intracranial infection were enrolled in the present study from January 2004 to December 2013.All patients with rare intracranial infections were confirmed by histopathologic examination; the misdiagnosis rate is 100% in the present series. The initial diagnosis included metastatic tumor, glioma, meningioma, cyst disease, hematoma, and epidermoid cysts. The features of magnetic resonance imaging were isointense (5/12), hyperintense (5/12), hypointense (2/12) on T1 weight, and hypointense (4/12) and hyperintense (8/12) on T2 weight, respectively, as well as nonspecific enhanced findings in the present study. The most common etiology of misdiagnosed intracranial infections was fungal abscess and neurocysticercosis that account for 25% (3/12) of the infections. Uncommon intracranial toxoplasmosis in 1 case and 1 case of cerebral alveolar echinococcosis in 1 case were also observed, respectively (8.3%). In addition, 2 cases (16.7%) of all patients were proved to have intracranial abscess with specific pathogen: one is Staphylococcus aureus, and the other is Serratia marcescens. Two intracranial abscesses (16.7%) with nonspecific pathogen were also found in the present study. The follow-up was conducted on all patients during a period of 3 to 96 months (average, 48 mo), of which 91.7% patients had a favorable outcome (Glasgow Outcome Scale 5 and 4) and 8.3% had an unfavorable outcome (Glasgow Outcome Scale ≤3).Primary diagnosis of rare intracranial infections should be emphasized; favorable outcome could be achieved by early microsurgical intervention and timely effective antibiotics.