We reviewed the clinical records of 51 extensively investigated pediatric patients with structural abnormalities of the cerebellum as revealed by magnetic resonance imaging (MRI). Ten had hypoplasia of the vermis, 21 had hypoplasia of the vermis and cerebellar hemispheres, 2 had pontocerebellar hypoplasia, and 18 had progressive cerebellar atrophy. A clear diagnosis was reached in 37 (72.5%). Initial characterization of the cerebellar alterations by MRI separated hypoplastic from atrophic cases and confirmed MRI as an essential preliminary means for distinguishing malformations from metabolic-degenerative conditions. However, the diagnostic possibilities are so numerous that it is not feasible to propose a standardized diagnostic protocol for pediatric patients with an altered cerebellum. Subsequent investigations should be suggested by the neuroradiologic and clinical peculiarities of each case.