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The neurological examination findings, clinical pathology (including Coccidioides immitis IgG/IgM serology) and magnetic resonance imaging (MRI) findings in 13 dogs with a novel form of intracranial coccidioidomycosis are described in a retrospective case series, with long-term clinical resolution documented in 11/13 dogs (84.6% of cases) with oral fluconazole therapy. The medical records of the Veterinary Neurological Center in Phoenix, Arizona from the years 2000 to 2017 were utilized to search for dogs with appropriate inclusion criteria. Magnetic resonance imaging findings were highly consistent across all cases and characteristically demonstrated bilaterally symmetric T2 hyperintensity throughout the frontal lobes, caudate nuclei, and rostral internal capsule, ±faint, wispy contrast enhancement on T1-postcontrast images. These findings were in stark contrast to previously reported MRI findings in dogs with intracranial coccidioidomycosis, which were typically characterized by a focal, strongly contrast enhancing granuloma and extensive vasogenic edema, typically unilateral. Schnauzer breeds represented eight of 13 (61.5%) cases, possibly suggesting a breed predilection. Three cases underwent repeat MRI after resolution of neurological signs and documentation of a decreased Coccidioides titer in response to fluconazole therapy. All demonstrated complete resolution of previously identified lesions, but with marked, severe atrophy of the caudate nuclei and frontal lobes bilaterally. Findings from this study document a variant appearance for intracranial coccidioidomycosis that, to the author's knowledge, has not been previously described in dogs. Authors propose that, although this variant demonstrates extensive pathological changes within the forebrain, the clinical outcome and response to treatment is favorable in a majority of cases., (© 2018 American College of Veterinary Radiology.)