Infantile desmoplastic ganglioglioma is a supratentorial superficially located cystic neuroepithelial tumor. It is an exceedingly rare tumor with an incidence of <0.1% of central nervous tumor and approximately 60 cases reported in the literature. We present a case of a 3-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 revealed a large solid and cystic mass in the temporal region measuring 8.6cm x 7.9cm x 5.1cm.  The infant underwent complete surgical resection and post-surgical pathology revealed a diagnosis of infantile desmoplastic ganglioglioma. 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.