A 22-year-old female presented with a calcifying pseudotumor of the neural axis manifesting as generalized convulsive seizure twice within 1 year. Computed tomography revealed a small, calcified mass lesion located in the right parietal lobe adjacent to the skull. The tumor was composed of an extensively calcified mass with accompanying peripheral epithelioid cells and focal mature bone structure, consistent with the diagnosis of a calcifying pseudotumor of the neural axis. Following complete excision of the tumor, the patient has been free from seizures for 8 years.