Painful Legs and Moving Toes syndrome (PLMT) is a rare neurological movement disorder characterized by involuntary and irregular movements of one or more toes, often together, with moderate to severe diffuse pain at the foot and leg. It can appear on one or both sides and in the upper limbs as well as lower limbs. The etiology of PLMT is still unclear; therefore, the main symptoms of PLMT, including pain in limbs as well as involuntary movements of toes, can be treated only symptomatically, and the outcomes are often unsatisfactory. Central or peripheral nerve injuries have been assumed to be the possible causes of irregular involuntary movements, often accompanied by chronic pain; however, its etiology is unknown in most cases. The management of PLMT is very difficult because of its rare prevalence and obscure pathogenesis, and there have been no clear guidelines for the treatment of PLMT. Oral medications (e.g., GABAergic agents, benzodiazepines, antiepileptics, antidepressants, atypical antipsychotics, beta-blockers, corticosteroids, cannabis, calcitonin, and analgesics) and several therapeutic interventions (e.g., local botulinum neurotoxin-A injection, spinal cord stimulation, anesthetic, and surgical interventions) have been recommended, based on individual experiences from several cases. Its etiology, pathogenesis, and effective treatments should be further discussed.