Ptosis is not a feature observed in amyotrophic lateral sclerosis (ALS). We describe two old women with bulbar-onset ALS and rapid progression, in whom ptosis and diplopia were noted. They did not improve on pyridostigmine or steroids. Antibodies against acetylcholine receptor were negative, thymoma was excluded, but neurophysiological showed marked neuromuscular transmission failure in orbicularis oculi. We discuss the association between ALS and ocular myasthenia gravis in these cases.