Corticobasal syndrome (CBS) is characterized by a slow progressive cognitive decline, apraxia, myoclonus, dystonia, and parkinsonism. We experienced a rapidly progressing CBS patient (onset to bed-ridden within 2 years) presenting only with resting tremor but showing complete unilateral loss of dopamine transporter binding. This case exhibited distinct FDG PET findings involving the unilateral severe anterior frontal cortex, caudate nucleus, and contralateral cerebellum, which is different from classical CBS. However, to date, no detailed serial functional imaging study has been performed in rapidly progressing CBS, so these FDG PET and CIT PET findings may help clinicians to recognize this fulminant type of corticobasal degeneration.