Herein, we will discuss in detail the available evidence of the physiological role of CHIP in the context of neurological diseases, such as intracerebral hemorrhage (ICH), ischemic stroke, Alzheimer’s disease (AD), Parkinson’s disease (PD), polyglutamine (PolyQ) diseases, and spinocerebellar ataxia autosomal recessive 16 (SCAR16) and spinocerebellar ataxia 48 (SCA48) caused by CHIP mutation (Fig. 1). The gene discussed is STUB1; the disease is Alzheimer disease.