Driving is important for employment, social activities, and for the feeling of independence. The decision to cease driving affects the quality of life and has been associated with reduced mobility, social isolation, and sadness. Patients with neurodegenerative disorders can experience difficulties while driving due to their cognitive, motor, and behavioral impairments. The aim of this review is to summarize the available literature on changes in driving competence and behavior in patients with neurodegenerative disorders, with a particular focus on Huntington's (HD), Parkinson's (PD), and Alzheimer's disease (AD). A systematic literature search was conducted in the PubMed/Medline database. Studies using on-road or simulated driving assessments were examined in this review. In addition, studies investigating the association between cognitive functioning and driving were included. The review identified 70 studies. Only a few publications were available on HD (n = 7) compared to PD (n = 32) and AD (n = 31). This review revealed that driving is impaired in patients with neurodegenerative disorders on all levels of driving competence. The errors most commonly committed were on the tactical level including lane maintenance and lane changing. Deficits in executive functioning, attention, and visuospatial abilities can partially predict driving competence, and the performance on neuropsychological tests might be useful when discussing potential driving cessation. Currently, there is no gold standard to assess driving ability using clinical measures such as neuropsychological assessments, so more studies are necessary to detect valid screening tools and develop useful and reliable evidence-based guidelines.