The presence of parkinsonism is required for the diagnosis of certain clinical syndromes and, together with demographic, clinical, and genetic features, can be highly predictive of underlying pathology, usually α-synuclein or tau aggregation. Nevertheless, parkinsonian features may be present in clinical syndromes not typically associated with parkinsonism and, therefore, complicate differential diagnosis. On the other hand, certain tauopathies have a variable frequency of associated parkinsonian disturbances, further challenging clinical to pathologic diagnostic accuracy. In this chapter, we briefly describe less-common clinical syndromes that may present with, or eventually manifest, extrapyramidal symptoms characteristic of parkinsonism but for which parkinsonism is not typically considered a core clinical feature. Although some of these clinical syndromes are primarily associated with tauopathies (frontotemporal degeneration with parkinsonism linked to chromosome 17, primary progressive apraxia of speech and traumatic encephalopathy syndrome), tauopathy is seldom the underlying pathology in cases of primary progressive aphasia.