Man-in-the barrel syndrome is a descriptive term of proximal dominant weakness for upper limb and shoulder girdle muscles. The patient is constrained in a barrel around the trunk, completely prohibiting upper limb movements. The most common cause of this syndrome is bilateral anterior watershed infarctions due to cerebral hypoperfusion of anterior and middle cerebral artery territory. Furthermore, polyneuropathies, motor neuron diseases, central pontine myelinolysis and cervical spinal cord lesions lead to this clinical presentation. Because of unfavorable prognosis of this syndrome in most cases, the treatable etiological determination of the causes is crucial.