The dopamine deficiency syndrome Parkinson's disease (PD) is characterized by tremor, rigidity, bradykinesia and reduced postural reflexes. Conditions with similar symptoms but other causes than PD (about 1/3 of cases) are called atypical parkinsonism. From a neuropathological perspective, PD is associated with loss of nigro-striatal dopaminergic neurons (related to motor symptoms) and accumulation of alpha-synuclein-containing Lewy bodies in the mid-brain. Nigro-striatal pathways may also be involved in atypical parkinsonism, but lesions in other parts of the brain/basal ganglions dominate. Parkinsonism may also be related to chronic cerebrovascular disease or use of drugs with extrapyramidal side effects.Articles retrieved from PubMed were reviewed to examine current knowledge on diseases other than PD that can induce parkinsonism.Diseases with atypical parkinsonism usually start more symmetric than PD and tremor is either not present or differs from the typical rest tremor seen in PD. An exception is corticobasal degeneration with very asymmetric symptoms. Other (plus-) symptoms in atypical parkinsonism are (early) falling tendency, dizziness upon change of position, coordination difficulties or early cognitive decline. The article describes the clinical characteristics of atypical parkinsonism, possibilities of improved diagnostics with supplementary examinations and alternative treatment strategies.