This study aims to understand the congenital malformation known as Microcephaly. The purpose is to verify the etiologies, diagnosis and classification of microcephaly in a superficial manner, and to present a neuropsychological profile of this syndrome through the existing relationship with Intellectual Disability. In addition to describing the main neuropsychological domains injured through this disorder, such as: memory, attention, executive function and language. The proposed problem is to identify whether there are levels of severity of this malformation and how they affect child development. The relevance of this study is given because, through a brief review of the literature, it favors a better understanding of what microcephaly is about. In the theoretical framework, it defines what is Microcephaly, Intellectual Disability, describes the cognitive functions and presents a neuropsychological profile of a child with intellectual disability, since it is common in microcephaly. The methodological procedure adopted was the bibliographical research which raised concepts of several authors of the studied areas. It concludes by stating that it s not possible to indicate a single neuropsychological profile, since the child born with such disorder may reveal different kinds of difficulties, or even none at all. It suggests a more in depth study on the subject, so that a program for therapeutic intervention of neuropsychological evaluation and rehabilitation is made, in such a way to allow for a better quality of life.