Neurocutaneous melanocytosis(NCM) is a rare phakomatosis. It is characterized by giant (>20 cm in diameter or occupying >2% of the body surface area) or multiple congenital cutaneous nevi and leptomeningeal melanocytosis. It is often associated with many smaller satellite nevi. Most patients with neurocutaneous melanosis presented in the first 2 years of life with neurologic manifestations of increased intracranial pressure, mass lesions, or spinal cord compression. A 8-year-old girl with multiple congenital giant hairy nevi on her back, trunk, and head underwent resection of skin lesions 4 times. There was no family history of a similar condition. On examination, the child was fully conscious and had no dysmorphic features. There were eleven well defined hairy, hyperpigmented nevi with regular boarders seen all over her body. Magnetic resonance imaging and biopsy revealed the malignant melanoma of right temporal lobe. Unfortunately in spite of lobectomy and ventriculo-peritoneal shunt, she expired 1month thereafter due to the cardiac arrest. Herein, we report a rare case of neurocutaneous melonosis.