Neurocutaneous Melanocytosis: Case Report of a Catastrophic Evolution

Case presentation: A 2-year-old boy with Dandy-Walker syndrome diagnosed by obstetric ultrasound, presented diffuse and large nevi at birth. He was submitted to endoscopic third ventriculostomy 15 days old due obstructive hydrocephalus. Spinal fluid then sent for analysis but showed no melanocytes. MRI of the brain additional findings mutation could not be performed. After 1 month he needed a ventriculoperitoneal shunt. During COVID-19 pandemic, lost follow-up care until presenting emergency room decreased level consciousness, respiratory distress flaccid paraparesis 21 months age. new revealed hyperintense signal which characterized an expansive lesion embracing bulb obliterating great cistern on T2 weighted images. biopsy performed showing leptomeningeal melanoma, therefore, confirming diagnosis neurocutaneous melanocytosis (NCM). As there were available curative options, palliative extubation