Rationale: Central neurocytoma occurring outside the ventricles is known as extraventricular neurocytoma (EVN). EVN is rare and its magnetic resonance imaging (MRI) findings vary greatly and overlap with the imaging features of other intracerebral primary tumors. Patient concerns: A 21-year-old woman with an intrauterine pregnancy of 18+2 weeks complained of dizziness and headache for 3 months. Diagnosis: A 8.6 cm × 5.8 cm × 3.7 cm space-occupying lesion was seen in the right frontal lobe on MRI, with mixed long signals on T1-weighted imaging and mixed slightly long signals on T2-weighted imaging, slightly hyperintense on T2-weighted imaging fluid attenuated inversion recovery images, and a large-scope long T1-weighted imaging and long T2-weighted imaging cystic component at the center of the lesion. A thick fence-like enhancement of the solid component at the periphery of the lesion was observed after injecting a contrast medium, while the internal cystic component was not enhanced. The MRI diagnosis was of glioma. The lesion was pathologically confirmed as an atypical central neurocytoma of the right frontal lobe. Interventions: Resection of the lesion and postoperative radiotherapy. Outcomes: The patient was lost to follow-up. Lessons: EVN can be considered as a differential diagnosis for lesions occurring in the cerebral hemispheres of young patients with cystic degeneration, thick fence-like enhancement, and peritumoral edema on MRI.