Magnetic resonance imaging (MRI) is the most sensitive imaging technique for assessing brainstem involvement in neurodegenerative diseases. An MRI can assess the degree of atrophy of the brainstem and alterations in signal intensity on T2-weighted images in the affected areas, which are the main imaging findings found in these diseases. Besides, diffusion-weighted MRI and proton MR spectroscopy are tools that play an important role in the characterization of these entities. Our purpose is to describe the neurodegenerative diseases that predominantly affect the brainstem highlighting the imaging findings most useful for diagnosis.