We report an intracranial leiomyosarcoma in the pontine cistern of a 34-yea
r-old woman infected with the human immunodeficiency virus (HIV), The clini
cal, radiological and pathological data are reviewed. The tumor was Epstein
-Barr virus (EBV) positive by in situ hybridization. This case emphasizes t
hat smooth muscle neoplasms arising in the setting of immunocompromise can
occur intracranially, and corroborates a hypothesis that EBV coinfection ma
y have a role in development of these tumors.