The syndrome of alexia without agraphia occurs rarely in multiple sclerosis (MS). We report a patient with right homonymous hemianopsia and alexia without agraphia as his initial manifestations of relapsing-remitting MS. Magnetic resonance imaging (MRI) demonstrated a hyperintense lesion in the left occipital subcortical white matter (WM) and an enhancing lesion in the splenium of the corpus callosum. The clinical presentation and MRI findings were consistent with disconnection of the functional right occipital visual cortex from structures responsible for language comprehension in the left hemisphere. The diagnosis of MS was confirmed by subsequent development of additional periventricular WM lesions.