Olivopontocerebellar atrophy (OPCA) is a rare progressive heterogeneous neurological condition characterized by neuronal loss in the inferior olives, ventral pons, and cerebellar cortex brain regions. Several sub-classifications of OPCA exist based on differences in inheritance patterns (familial and sporadic) and age of onset (infantile, juvenile, and adult onset), a variety that suggests multiple underlying etiologies resulting in similar clinical presentation and neuropathology.