The gene locus for neurilemmomatosis has been reported to Lie within t
he neurofibromatosis type 2 gene region, suggesting that these 2 disea
ses may be identical. We describe an adolescent girl with multiple neu
rilemmomas and juvenile xanthogranulomas, who was found to have bilate
ral acoustic neuromas and multiple central nervous tissue tumors as se
en in patients with a diagnosis of neurofibromatosis type 2. This case
and recent genetic studies suggest that neurilemmomatosis and neurofi
bromatosis type 2 are the same disease.