Clinical consequences of hypomagnesemia are manifold and include various neurological syndromes up to life-threatening conditions. Despite its importance, magnesium is generally not routinely determined leading to an under-recognition of hypomagnesemia-related disorders. In the past years, there are growing numbers of reports of hypomagnesemia-induced cerebellar syndromes (HiCS) with corresponding cerebellar edema, which might be a distinct disease entity. To provide further insights into HiCS, we describe a patient with HiCS and performed a literature review on cerebellar syndromes due to severe hypomagnesemia with regard to the clinical, MRI, and laboratory findings. We identified 17 cases, so including our case, 18 cases contribute to this review. Summarized, HiCS seems to be a distinct disease entity because of the remarkable similarities of clinical, MRI, and laboratory features. It should be diagnosed and treated early to avoid recurrent disease courses, residual symptoms, and potentially life-threatening conditions such as seizures. Physicians must be alert to HiCS as magnesium is usually not part of the routine electrolyte panel.