Introduction: Leptomeningitis and pachymeningitis are known to occur consecutive to many causes.
Observation: We report the case of a 24-year-old woman with symptoms of raised intracranial pressure and repeated switching transient hemiparesis. The magnetic resonance imaging (MRI) showed a pachyleptomeningitis. Search for a cause was negative. The pathology examination of meningeal tissue revealed a malignant melanoma, without any sign of cutaneous melanoma, leading to the diagnosis of primary leptomeningeal malignant melanoma.
Conclusion: A meningeal biopsy can enable the rare diagnosis of primary leptomeningeal malignant melanoma in a patient with unexplained pachyleptomeningitis.
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