Sneddon syndrome is a non-inflammatory, non-atherosclerotic disease involving small and medium-sized arteries of the brain and of the skin. The arteriographic examination is often negative despite progressive impairment of the neurological status. In 3 patients with Sneddon syndrome cerebral perfusion was assessed with single photon emission computerized tomography (SPECT) and technetium99 m-hexamethylpropylenamineoxime (Tc99 HM PAO). A correlation between clinical and SPECT finding was found, with significant focal reduction of regional cerebral blood flow (rCBF) in the two more severely affected patients.