Here, we report the case of a 3-year-old boy with a de novo splicing variant in CEP85L. He presented with mild neurodevelopmental delay and a brain magnetic resonance imaging (MRI) showing temporo–parieto–occipital prevalent lissencephaly with subcortical band heterotopia, a combination not reported before in CEP85L patients. This evidence concerns the gene CEP85L and lissencephaly spectrum disorders.