Although syringomyelia has been associated with Horner's syndrome, it is ty
pically associated with other neurological findings such as upper Limb weak
ness or numbness. A patient is described who had an isolated Horner's syndr
ome as the only manifestation of syringomyelia. A 76 year old woman was dis
covered to have right upper lid ptosis and right pupillary miosis. Neurolog
ical examination was unremarkable, and pharmacological testing was consiste
nt with localisation of the lesion to a first or second order sympathetic n
euron. Neuroimaging disclosed a Chiari I malformation with a syrinx extendi
ng to the C2 to C4 level. An isolated Horner's syndrome may be the presenti
ng manifestation of syringomyelia.