Meningiomas frequently lose parts of chromosome 22 (CHR 22), suggesting that a meningioma tumor-suppressor gene is located on CHR 22. Since meningiomas are common in neurofibromatosis 2 (NF2) and the NF2 gene is mapped to CHR 22, the NF2 gene is a candidate for the meningioma gene. To determine whether NF2 and familial meningioma are allelic mutations, we studied a family with multiple meningiomas and ependymomas in two generations using genetic linkage analysis with DNA markers known to flank the NF2 locus. Multipoint linkage analysis resulted in location scores < -2 for a region of 15 cM including the NF2 region. These results support the existence of a familial meningioma locus that is distinct from the NF2 locus.