Granulomatous amebic encephalitis (GAE) is an exceedingly rare disease with a mortality rate of over 90%. It can be caused by species such as Balamuthia mandrillaris, Acanthamoeba, or Sappinia pedata. Unlike primary amebic meningoencephalitis (PAM), which typically presents with an acute progression resulting in multiple lesions, GAE often follows a subacute to chronic course and is typically associated with a single space occupying lesion. Environmental exposures, including water, soil, and dust, are potential risk factors for amebic inoculation. Our case illustrates a novel presentation of amebic encephalitis that was misdiagnosed as herpes simplex virus (HSV) encephalitis.