Neurocutaneous melanosis (NCM) is a rare neuroectodermal dysplasia that includes both central nervous system (CNS) and integumentary melanocytic abnormalities. NCM can present with varied clinical and imaging findings, classically presenting with large melanocytic nevi involving the posterior axial trunk region. We describe a case with predominant small non-scalp and non posterior axial-trunk nevi, which nevertheless demonstrated typical CNS imaging findings. Our case clinically presented with epilepsy that was controlled by medical treatment. The patient also demonstrated motor developmental delay, and an otherwise stable course.
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