Desmoplastic infantile ganglioglioma (DIG) is a supratentorial superficially-located cystic neuroepithelial tumor. It is an exceedingly rare tumor with an incidence of <0.1% of central nervous tumors; approximately 60 cases have been reported in the literature. We present a case of a three-month-old infant with progressive disordered movements described as intermittent upper body stiffening with associated eye blinking, drooling, and change in level of alertness. A seizure was witnessed in the emergency department, after which the child was sent for imaging studies. Magnetic resonance imaging (MRI) revealed a large solid and cystic mass in the temporal region measuring 8.6cm × 7.9cm × 5.1cm. The infant underwent complete surgical resection, and post-surgical pathology revealed a diagnosis of DIG. The patient had an excellent post-operative course in the months following discharge. At his last well-child visit, no neurological deficits were appreciated and the infant was meeting expected milestones for his age.