Hemiparkinsonism-hemiatrophy syndrome (HPHA) is a rare form of parkinsonism, characterized by unilateral parkinsonism, ipsilateral body atrophy and early age of onset. We report a 59-year-old woman with hemiatrophy of her face and upper limb on the left side presenting progressive hemiparkinsonism. Most of the HPHA patients have early age of onset, however, HPHA patients with age of onset over 50 years old have been reported. The clinical features of CBD are partly similar to those of HPHA, therefore it is important to consider HPHA as differential diagnosis of CBD even if the onset is late. In our patient, hemiatrophy was useful to differentiate HPHA from CBD. In previous reports, there was a difference between the dopamine D2 receptor binding capacity of CBD and that of HPHA. While the dopamine D2 receptor binding capacity of CBD was decreased asymmetrically, that of HPHA was not decreased. The PET finding of our patient revealed that asymmetrical reduction of [11C]-CFT uptake, meaning unilateral dopaminergic presynaptic hypofunction. However, [11C]-raclopride uptake, which assesses dopamine D2 receptor binding capacity, was normal. These PET findings are consistent with previous reports on HPHA. In our patient, hemiatrophy and PET findings were useful to diagnose HPHA.