Objectives Guillain-Barré syndrome (GBS) is an acute clinical syndrome that is classically featured by rapidly evolving motor weakness, mild sensory loss and hypo- or areflexia. Uncommon variants such as pure sensory phenotype and restricted dysautonomia have been described in recent years.

Case We report a case of rare variant of GBS consisting of unilateral cranial neve X dysfunction and profound postural hypotension. Clinical diagnosis of GBS is supported by areflexia, abnormal sensory findings on electrophysiological study and CSF cytoalbuminologic dissociation.

Conclusions The combination of cranial nerve and autonomic involvement is a rare variant of GBS. Early recognition of uncommon variants of GBS can be challenging and definitive diagnosis is not always possible using the current diagnostic criteria for typical syndrome.