We report a patient, who developed bilateral ophthalmoplegia and ataxia, with weakness and numbness of upper extremities and later also weakness of lower extremities, accompanied by drowsiness with hyperreflexia. Based on clinical and EMG findings, high positivity of anti-GQ1b antibodies and prompt treatment response to intravenous immunoglobulins, we conclude that the final diagnosis of our patients is BBE overlapping with GBS.