Variant forms of the Guillain-Barré syndrome are characterized by their localized or regional involvement of the peripheral and autonomic nerves. As there is no single clinical or serologic marker for Guillain-Barré syndrome, diagnosis of this condition is based upon consistent clinical, laboratory, and neurophysiologic findings, with exclusion of other conditions mimicking this disorder. Recognition of atypical cases enables anticipatory monitoring for disease complications and identifies therapeutic options for affected children. A regional variant with predominant facial, neck, and arm weakness without sensory loss has been reported in adults but only rarely described in childhood. This study reports clinical and neurophysiologic findings in two children with the pharyngeal-cervical-brachial form of Guillain-Barré syndrome. These are the youngest cases of this uncommon disorder reported to date.