Meningeal melanomatosis is an extremely rare and aggressive melanocytic neoplasm of the central nervous system characterized by diffuse leptomeningeal involvement. Its occurrence during pregnancy is exceptionally uncommon and presents significant diagnostic and therapeutic challenges. A 39-year-old woman at 15 weeks of gestation presented with progressive headache and left-sided weakness. Brain MRI revealed a right frontal extra-axial mass with surrounding edema and subfalcine herniation. Surgical resection was performed due to neurological deterioration. Histopathological and immunohistochemical analysis confirmed malignant melanoma (S100-positive, HMB-45-positive, high Ki-67 index). Postoperative MRI demonstrated residual extra-axial mass suspicious for meningeal melanomatosis. Focal radiotherapy (20 Gy) was administered. The patient subsequently experienced spontaneous preterm delivery. Four months later, bilateral breast metastases were identified. Progressive neurological decline occurred, and the patient died eight months after initial diagnosis.Meningeal melanomatosis during pregnancy represents a rare and highly aggressive condition with poor prognosis despite multimodal treatment.

Keywords: Meningeal melanomatosis; Pregnancy; melanoma