Cerebellar hypoplasia is characterized by reduced cerebellar volume, even though cerebellar shape is (near) normal. It consists of a heterogeneous group of disorders of cerebellar maldevelopment presenting as early-onset non–progressive congenital ataxia, hypotonia and motor learning disability. Various causes have been incriminated, including hereditary, metabolic, toxic and viral agents. It was first reported by French neurologist Octave Crouzon in 1929. In 1940, an unclaimed body came for dissection in London Hospital and was discovered to have no cerebellum. This unique case was appropriately named "human brain without a cerebellum" and was used every year in the Department of Anatomy at Cambridge University in a neuroscience course for medical students. Cerebellar hypoplasia can sometimes present alongside hypoplasia of the corpus callosum or pons. It can also be associated with hydrocephalus or an enlarged fourth ventricle; this is called Dandy–Walker malformation. Signs and symptoms Non-progressive early onset ataxia and poor motor learning are the most common presentation. Diagnosis Magnetic resonance imaging Three dimensional (3D) T2-weighted (T2w), axial, coronal, sagittal magnetic resonance imaging (MRI) is considered appropriate for differentiation between gray matter and white matter acquisition of high-resolution anatomic information. T2w, axial and coronal imaging is more suitable for acquisition of high-resolution anatomic information and delineation of cortex, white matter, and gray matter nuclei. Diffusion tensor, axial imaging is used for evaluation of white matter microstructural integrity, identification of white matter tracts. CISS, axial + MPR imaging for evaluation of cerebellar folia, cranial nerves, ventricles, and foramina. Susceptibility weighted axial scans are employed for identification and characterization of hemorrhage, blood products, calcification, and iron accumulation. Classification Classification systems for malformations of the cerebellum are varied and are constantly being revised as greater understanding of the underlying genetics and embryology of the disorders is uncovered. A classification proposed by Patel S in 2002 divides cerebellar malformations in two broad groups; those with cerebellar hypoplasia and; those with cerebellar dysplasia. I. Cerebellar hypoplasia A. Focal hypoplasia 1. Isolated vermis 2. One hemisphere hypoplasia B. Generalized hypoplasia 1. With enlarged fourth ventricle (“cyst,”), Dandy–Walker continuum 2. Normal fourth ventricle (no “cyst”) a. With normal pons b. With small pons i. Normal foliation a) Pontocerebellar hypoplasias of Barth, types I and II b) Cerebellar hypoplasias, not otherwise specified Treatment There is no standard course of treatment for cerebellar hypoplasia. Treatment depends upon the underlying disorder and the severity of symptoms. Generally, treatment is symptomatic and supportive. Balance