Giant axonal neuropathy (GAN) is a neurodegenerative disorder classified within the hereditary motor and sensory neuropathies, affecting both the peripheral and central nervous systems. GAN typically presents in early childhood before the age of five years and progresses to death usually by early adulthood. Various imaging findings in giant axonal neuropathy have been described and documented in literature in the form multiple case reports. We here present a pictorial essay of all the major imaging GAN findings described in the literature. In addition, involvement of the dentate nucleus hitherto not described in the literature was noted in the present case.