Background: Meningeal melanocytoma was first described over 30 years ago as a benign tumour derived from melanocytes. Since then, data suggest that its mode of presentation is variable without a clear predilection for any particular site in the neuroaxis. Although classified as a benign tumour, this tumour shows a marked tendency towards reduced survival following subtotal resection and transformation over time in a limited number to malignant melanoma. Incomplete resection of these tumours without postoperative radiotherapy has only a 42% 5-year survival rate. Its classification as a benign tumour should be revised, given the published 5-year survival data.
Illustrative case: We report a fatal case of meningeal melanocytoma in the cerebello-pontine angle in a 10-year-old child. This case exemplifies the vascular nature of these lesions even with minimal vascular blush on angiography. An updated literature search is presented, the results of which highlight the need for close follow-up and adjuvant treatment following subtotal resection.