Rosette-forming glioneural tumors (RGNTs) are rare. Here, we report a case of RGNT of the fourth ventricle in an 18-year-old female. The patient presented with a 4-month history of headache and dizziness. Neurological examination showed papilledema, impaired tandem gait, and right-sided dysmetria. Radiological images showed a posterior fossa lesion in the fourth ventricle with hydrocephalus. An emergent ventriculostomy was performed followed by gross total surgical resection of the lesion. Histopathological examination confirmed the diagnosis of RGNT. The patient developed posterior fossa syndrome postoperatively which improved on follow-up. Although rare, RGNT should be considered in the differential diagnoses of posterior fossa lesions in young patients. Given its benign course, surgical resection remains the treatment of choice.
