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Primary diffuse leptomeningeal melanomatosis is a rare and very aggressive neoplasm with only 6 pediatric cases reported so far. Recognizing this condition is unfortunately difficult due to the nonspecific clinical presentation. Nevertheless, imaging may help in a timely diagnosis that can better direct the management of these patients, especially in the light of the rapidly ominous course of the pathology. We describe three cases of pediatric primary diffuse leptomeningeal melanomatosis and review the literature regarding this entity, focusing on computed tomography and magnetic resonance imaging findings.