Miller Fisher syndrome (MFS) is a variant of Guillain-Barre syndrome (GBS) characterized by acute onset of ophthalmoplegia, ataxia and areflexia. Diplopia, altered ocular motility, pupillary dysfunction and blepharoptosis have been reported in MFS patients. However, the relationship between antiphospholipid antibody and MFS remains largely unclear. Here we report the first Chinese patient with MFS who showed proptosis and pain and had serum anti-GQ1b antibodies and IgM anticardiolipin antibody. A 64-year-old woman presented with numbness of distal limbs, proptosis and pain after upper respiratory tract infection. Her bilateral eye pain got exacerbated, along with blepharoptosis, ophthalmoplegia and horizontal binocular diplopia. Ophthalmological exam revealed normal pressure in both eyes. The neurological examination revealed bilateral ptosis, proptosis with serious tenderness, ophthalmoplegia with dull pupil reaction to light, and areflexia. There was no ataxia. Serum anti-GQ1b antibodies and IgM anti-cardiolipin antibody were positive. Our case suggests that MFS presenting with proptosis and pain may be associated with serum anti-GQ1b antibodies and IgM anti-cardiolipin antibody.