BACKGROUND: In the setting of a normal neurological examination, accommodation spasm is frequently attributed to a non-organic etiology. Occasionally, organic disorders are associated. In particular, central lesions involving the dorsal midbrain and quadrigeminal plate have been described with disorders of accommodation. HISTORY AND SIGNS: A 36-year-old woman with idiopathic intracranial hypertension (IIH) had visual blur from pseudo-myopia due to accommodative spasm. Magnetic resonance imaging (MRI) revealed a pineal cyst that was reported to be an incidental finding. The patient had persistent papilledema and recurrent episodes of unilateral and bilateral visual blur from accommodative spasm despite medical management. THERAPY AND OUTCOME: A lumboperitoneal shunt effectively lowered her intracranial pressure (ICP). Thereafter, all symptoms of increased ICP, accommodative spasm and papilledema resolved. A functional, non-organic cause for accommodation spasm was not suspected. CONCLUSIONS: To our knowledge, this is the first report of isolated accommodative spasm as a presenting symptom in a patient with IIH. The patient's accommodative spasm resolved with lowering of the ICP. It remains speculative whether her pineal cyst played a role in triggering the accommodative spasm.