Although there have been reports regarding the frequent involvement of the pupils in Miller Fisher syndrome (MFS) and related syndromes, internal ophthalmoplegia has not been described as the initial sole manifestation of typical MFS. Recently, we encountered a woman with MFS whose initial manifestation was blurred vision because of bilateral tonic pupils. Investigations, including brain imaging, cerebrospinal fluid examination and nerve conduction studies were normal except for elevated serum levels of immunoglobulin G anti-GQ1b antibody. During the disease, she subsequently developed bilateral sixth and seventh nerve palsies, gait ataxia, and areflexia. We treated her with intravenous immunoglobulin and she showed a stepwise recovery from her illness. This case suggests that bilateral internal ophthalmoplegia can be a preceding sole manifestation of typical MFS.