Background: Hemimegalencephaly (HMC) is a disorder associated with enlarged and dysplastic hamartomatous overgrowth of all or part of the one cerebral hemisphere that can be isolated or associated with other syndromes. In the normal development of the brain it is important to bear in mind that there are two main processes: firstly the development of the hemispheres and the corpus callosum, and secondly the cortical formation with proliferation, migration and organization of the cortex, which occurs mostly between 12 and 20 weeks of gestation. Case Report: We present a 22-week-old fetus with macrocephaly depending on HMC and emphasize the possibility of an early ultrasound diagnosis, the correlation in the diagnosis between 2D and 3D ultrasound, and the use of magnetic resonance imaging as an imaging method for a more precise diagnosis of neuronal migration anomalies. Conclusion: The diagnosis of HMC is possible at the time of the anomaly scan. The use and correlation with other diagnostic tools provide essential information for parent counseling in these complex cases.