A variant of Guillain-Barré syndrome (GBS) with predominant manifestation of facial diplegia (FD) has been described recently. This study aimed to characterize and determine the incidence of this FD-predominant GBS variant. The clinical and serological information of 900 consecutive patients were reviewed. In total, eight patients were identified between January 2007 and December 2010 as having FD accompanied by some features of GBS. These features were subjective sensory symptoms such as distal paresthesia (7/8, 88%), albumin-cytological (A/C) dissociation (7/8, 88%), antecedent infection (6/8, 75%), and minor nerve conduction study (NCS) abnormalities (5/7, 71%). One patient presented with the typical NCS feature of demyelinating neuropathy. Only two patients exhibited areflexia (2/8, 25%). None of the patients possessed any anti-ganglioside antibodies; however, the serum of two patients was positive for anti-mycoplasma antibody (2/6, 33%). FD variant of GBS occurred in less than 1% of our dataset. FD can be a regional variant of GBS when it is accompanied by supporting features, such as subjective tingling, A/C dissociation, and minor NCS abnormalities.