ABSTRACT
We report a case of congenital giant pigmented nevus with malignant melanoma of brain in a 14-year-old male patient. He had giant pigmented nevus on the back and neck, and multiple satellite lesions over the whole body since birth. One year prior to visit to our hospital, the patient suffered from various neurologie symptoms including headache, nausea, vomiting, seizure and right side motor weakness. Flistologic findings of skin lesions were benign nevocytic nevi. Computed tomogram of brain demonstrated increased densities in the both fronto-parietal leptameninges and brain parenchyme. Histologic findings of brain parenchyme by stereotaxic long needle biopsy showed the infiltration of melanin containing atypical melanocytes. There was no evidence of malignant melanoma at other organs. All of these findings suggested that origin of malignant melanoma of brain parenchyme was leptomeninges rather than skin.