Transient binocular diplopia is a frequent complaint in an everyday ophthalmic practice. Since the objective clinical examination is most often within normal limits between the diplopia attacks, a thorough history is mandatory to determine the possible causes and precipitating factors. Although transient diplopia can herald visual or even life-threatening associated conditions, it is most often related to benign diseases. An appropriate work-up depends on the history and the clinical evaluation, targeting specific underlying causes.