Guillain-Barré syndrome is clinically characterized by acute onset of generalized, symmetrical, and ascending muscle weakness and areflexia from peripheral nerve involvement. In Guillain-Barré syndrome variants, however, some patients have unusual distribution of muscle involvement. Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is characterized by oropharyngeal, neck, and upper limb muscle involvement. Although Guillain-Barré syndrome is one of several post-infectious diseases that cause limb muscle weakness, the incidence of pharyngeal-cervical-brachial variant is relatively low. Here we report the case of a 16-month-old boy who developed a rare form of Guillain-Barré syndrome, the pharyngeal-cervical-brachial variant of the disease. We concluded that taking all the other etiologic reasons into consideration, pharyngeal-cervical-brachial variant of Guillain-Barré syndrome should be remembered in patients with symptoms of bulbar and upper extremity weakness not only for early diagnosis but also to plan the treatment early and follow up the potential complications.