We present the unusual neuroimaging findings of focal cerebral leptomeningeal enhancement in association with corticopial calcifications, underlying a parietal convexity lipoma in 2 pediatric patients with normal psychomotor development, unremarkable physical findings, and recent onset of epilepsy. To our knowledge, this clinical and neuroradiological association has not been previously reported and shows overlapping features with Sturge-Weber syndrome without facial nevus and encephalocraniocutaneous lipomatosis, thus possibly representing an extremely rare variant within the spectrum of these congenital neurocutaneous disorders.