Two distinct forms of rabies—furious and paralytic—are recognized in humans. Diagnosis of the classical furious (encephalitic) form, which constitutes about 80% of human rabies cases, is based on its distinctive clinical signs and symptoms and rarely poses diagnostic difficulties. However laboratory assistance may be required in some cases wherein characteristic clinical features like aerophobia or hydrophobia are lacking. In clinical practice, the paralytic or atypical forms, which constitute about 20% of human rabies cases, pose a diagnostic dilemma. These cases are often clinically indistinguishable from Guillain-Barre syndrome (GBS) and also need to be differentiated from neuroparalytic complications due to Semple-type antirabies vaccine which is still being used in few countries like Mongolia, Myanmar, and Pakistan [8–11]. The situation is further compounded by lack of history of animal bite, psychiatric or other atypical clinical manifestations, unavailability of a definitive diagnostic test for GBS, and limited availability of tests for antemortem diagnosis of human rabies [9].