We have seen 32 patients with "apraxia of lid opening" (ALO) in the following clinical settings: as an isolated condition (3 patients), idiopathic blepharospasm (BSP, 20 patients, including 4 familial cases), progressive supranuclear palsy (PSP, 7 patients), and dystonic parkinsonian syndrome (2 patients). Twenty-nine patients treated with botulinum toxin into the orbicularis oculi muscle were rated before and after treatment and 83% of the patients improved on a clinical scale. Best results were obtained with injections directed toward the junction of the preseptal and pretarsal parts of the palpebral orbicularis oculi. Several patients also improved on anticholinergic drugs. Besides medical treatment, lid crutches, in conjunction with botulinum toxin injections, were useful in some patients. ALO is not a true apraxia; it constitutes an eyelid dystonia as shown by its clinical and electrophysiological features as well as pharmacological reactions and is encountered in a clinical spectrum ranging from an isolated form to predominant BSP. It was an important cause of treatment failures in botulinum toxin-treated BSP but by modifying our injection strategy and by adding anticholinergic drugs and also lid crutches, we obtained a good functional benefit.