Corticobasal syndrome (CBS) is characterized by asymmetric parkinsonism, apraxia, cortical sensory deficits, dystonia, myoclonus, and cognitive dysfunction. Although it was originally described as the clinical manifestation of corticobasal degeneration (CBD), it is now recognized that CBS may be the clinical presentation of a variety of neurodegenerative pathologic processes including, but not limited to, CBD, Alzheimer disease (AD) and frontotemporal lobar degeneration (FTLD). In order to evaluate the neuropathological correlates of CBS in our center, 37 retrospectively identified cases with clinical CBS and post-mortem examination were analyzed. CBD was the primary pathological diagnosis in only eight cases (22% of the total cohort), whereas the most common underlying pathology was AD with or without Lewy body disease (LBD) in 16 cases (43%). The remaining 35% of cases were found to have progressive supranuclear palsy (N=5), FTLD-TDP (N=3), LBD (N=2), unclassifiable tauopathy (N=1), mild developmental abnormality (N=1) and neuronal intranuclear inclusion body disease (N=1). Moreover, 27% of the CBS cases had other pathological findings in addition to the main neurodegenerative process, most often cerebrovascular disease and/or mild AD-related pathology. These findings confirm and illustrate the heterogeneous pathological substrate for CBS.