A 32.5-year-old female hybrid orangutan (Pongo pygmaeus) developed hind-limb stiffness that progressed to tetraparesis over 2 wk. Repeated diagnostic evaluations, including serial magnetic resonance imaging of the central nervous system, revealed nonspecific lesions involving both the deep white and gray matter with an intact blood–brain barrier. Multiple empirical treatments failed to produce improvement and the animal was humanely euthanized. Histology of a granuloma in the ileum contained a nematode parasite, most consistent with Baylisascaris procyonis. Additionally, neuropil vacuolization, rarefaction, astrocytic scarring, and an eosinophilic granuloma and lymphoeosinophilic perivascular cuffing in the brain were suggestive of nematode migration. These findings confirm the presence of visceral larval migrans and support the presence of neural larval migrans. This case report of Baylisascaris procyonis confirms the presentation for the first time in an ape and documents the difficulty in antemortem diagnosis of neural larval migrans.