Introduction: Papillary Glioneuronal Tumor (PGNT) is a rare tumor with approximately 70 cases reported in the last decade. This tumor is more common in young adults and is the most common clinical manifestation of headaches and seizures. This tumor has an indolent clinical behavior. Regarding gender prevalence there is no detailed epidemiological information available, because of the rarity and newness of this tumor.

Case Presentation: A 32-year-old female patient with a sudden headache and blurred vision referred to our hospital. The magnetic resonance imaging (MRI) revealed a heterogeneous mass with dimensions of 35 × 33 × 20 mm in the right frontal lobe. Histologically, a biphasic tumor composed of a glial component arranged in papillary architecture overlaying hyalinized vessels, associated with interpapillary areas, containing monotonous oligodendrocyte-like and ganglion-cells were seen. The immunohistochemical staining indicated glial fibrillary acidic protein (GFAP)-positive glial cells and positive synaptophysin and neuron-specific enolase (NSE) in interpapillary neurocytes.

Conclusions: Papillary Glioneuronal tumor is a rare tumor with slow growth and low grade. The possibility of transformation to high-grade tumor, is low. Due to having the structure of pseudopapillary, along with clinical and radiographic characteristics, it must be distinguished from other variants of glioneuronal tumor mix.