A 14-month-old previously healthy boy developed progressively worsening neurological symptoms secondary to eosinophilic meningoencephalitis with myelitis caused by raccoon roundworm (Baylisascaris procyonis) infection. MRI demonstrated T2 hyperintensity and enhancement of the cerebral white matter, cerebellum and spinal cord. Prior case reports have described signal abnormality within the brains of patients with raccoon roundworm neural larva migrans (NLM). This is a unique case in which spinal cord involvement was established by imaging. Knowledge of this combination of imaging findings expands the known imaging phenotype of this noteworthy infection.