A post-mortem examined case of cerebellar agenesis associated with microcephaly, agyria and deformities of the extremities and of the external ear in a newborn is presented. Such an association has not yet been reported in the literature. This is the seventh case of cerebellar agenesis ever registered in the literature. This case is compared with other previously published cases and the possible etiological factors involved as well as the period during which the cerebral malformations appeared in the present case are commented upon.