We present 2 unrelated patients found to have tumors of neural tissue origin, a neurothekeoma and a meningioma, who were additionally diagnosed with the syndrome of Birt-Hogg-Dubé (BHDS). We are unaware of previous BHDS patients with neural tissue tumors. In light of recent linkage analysis studies delineating the genetic susceptibility locus for BHDS, we speculate about a possible association between BHDS and neural tissue tumors.