Agenesis of the corpus callosum is an anomaly that may occur as isolated or in association with other central nervous system or systemic malformations. We report the case of an infant antenatal diagnosed with ventriculomegaly referred in the postnatal period to our department for imaging evaluation. Ultrasonography showed the absence of the corpus callosum and an interhemispheric lesion highly suggestive for a cerebral lipoma. The diagnosis was confirmed through MRI.