Spinal and bulbar (bulbospinal) muscular atrophy (BSMA, SBMA, Kennedy's disease) is a progressive motor neuron disease with rare involvement of structures other than the lower motor neuron, such as the endocrine system and the central nervous system (CNS). Aim of the review was to study type and frequency of clinical, imaging, and functional (CNS) abnormalities in SBMA patients. The most frequent clinical CNS manifestations in SBMA are postural or kinetic tremor predominantly of the hands and mild cognitive impairment. The most frequent instrumental CNS abnormality in SBMA patients are white matter lesions, visible on voxel-based morphometry, magnetic resonance spectroscopy, or diffusion tensor imaging. Single patients with enlarged pituitary volume, or diminished somato-sensory representation in the cortex have been also reported. Seizures, epilepsy, ataxia, spasticity, dystonia, or migraine have not been found in SBMA patients. Only supportive treatment is available for CNS manifestations in SBMA. It is concluded that the most frequent CNS abnormalities in SBMA are tremor, cognitive impairment, and white matter lesions on new imaging modalities. CNS involvement in SBMA should not be neglected as a phenotypic manifestation of SBMA and, apart from cognitive involvement, may help to differentiate clinically SBMA from other types of motor neuron disease.