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Leptomeningeal melanocytosis is a rare cause of seizure in the pediatric population. Shown here is a case of this disease in a 9-year-old male who presented with seizures and minor trauma. Imaging showed progression of leptomeningeal enhancement in the setting of increased seizure activity, and biopsy confirmed the diagnosis. The patient received immunotherapy but eventually succumbed to the disease. This case serves as an educational tool to improve awareness of melanocytic proliferation as a differential consideration for leptomeningeal enhancement.

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