Lewy bodies (LBs) were observed in the giant pyramidal cells of Betz in the motor cortex of a patient with Parkinson's disease (PD) of 8-year duration. The patient had shown typical clinical features of PD. No dementia or pyramidal tract signs had been observed. The LBs, found in 8 of 747 Betz cells counted (1.1%), appeared as homogeneous or laminated, spherical inclusions with a clear surrounding halo and were strongly immunoreactive for alpha-synuclein. To our knowledge, this is the first demonstration of LBs in Betz cells. Considering the significant loss of neurons in the predilection sites for LBs, it is possible that in this patient, the motor cortex was also involved in the disease process of PD.