The purpose of this study was to investigate spontaneous low-frequency fluctuations in CADASIL patients during resting-state fMRI scans. Eleven patients (aged, 33-66 years, 6 female) and 11 age- and gender-matched healthy controls were recruited. Amplitude of low-frequency fluctuation (ALFF) was calculated to measure spontaneous brain activity. The results showed that CADASIL patients exhibited significantly decreased ALFF in the bilateral precuneus, and increased ALFF in the midbrain/ pons, the insula/ temporal pole, and the anterior cingulate gyrus/ corpus callosum. Our study first provides empirical evidence for altered spontaneous neuronal activity in CADASIL patients.