Primary biliary cholangitis (PBC) is a rare chronic disease, characterized by progressive cholestasis that could end in end-stage liver disease. Its diagnosis is based in the presence of a cholestatic pattern and antimitochondrial antibodies. Neurological complications of PBC are unusual, but there are descriptions of association with myopathies and neuropathies, being polymyositis the most frequent. We report two cases of patients with the diagnosis of PBC with neurologic complications: one case with asymptomatic PBC and myopathy and another one with demyelinating neuropathy. Neurologic diseases in patients with PBC have been described mainly as case reports. The co-occurrence of both entities suggests the possible existence of a common pathogenic mechanism but the real etiopathogeny is still unknown.