Cortical dysgenesis (CD) is a frequent inherited brain malformation. CD is a key cause of epileptic syndrome in children. In this review, the author presents a current classification of CD, etiological factors of their development, including gene mutations and adverse effects of various toxins and perinatal factors. A spectrum of clinical symptoms of CD with epileptic seizures is discussed in details. A role of current MRI-regimes in the diagnosis, the management of patients and prognosis of the course of CD are highlighted.