Currently we do not yet know the populational burden of work-related and occupational neurological diseases. There is, however, scientific evidence for correlations between chemical, biological, and physical agents at workplace and different acute, subacute, and chronic neurological diseases and injuries. Exposure to pesticides and manganese is a risk factor for development of parkinsonism. Inhalation of diesel fumes, especially in professional drivers, increases the risk for development of motor neuron diseases. Repetitive mild subcommotion brain and spinal cord injuries can lead to chronic traumatic encephalopathy which can manifest itself as dementia, Parkinson’s disease or motor neuron disease. Many work-related neurological diseases, especially neurodegenerative ones, develop after a period of many years or even decades after exposure to toxic and harmful agents. Because we still lack efficacious treatment for neurodegenerative diseases, it is crucial to know and be aware of these harmful agents, so as to be able to develop successful preventive strategies and thus lower the burden of these diseases.