ABCA2, which belongs to the A subclass of the ATP-binding cassette (ABC) transporter superfamily, is predominantly expressed in the cytoplasm of oligodendrocytes and Schwann cells, the myelin-forming cells in brain and peripheral nerve, respectively. Here, we demonstrate by immunoblot and immunohistochemistry that ABCA2 is expressed in benign vestibular schwannomas, which contain neither axons nor compact myelin. The expression patterns of ABCA2 in combination with other markers showed phenotypic heterogeneity in schwannomas. The majority of cells in fascicular Antoni type A areas coexpressed ABCA2, Ca2+-binding protein S100beta, and p75 nerve growth factor receptor. In contrast, considerably varied expression levels of ABCA2 and p75 were more prominent in hypocellular type B areas than in type A areas. These data suggest that ABCA2 may be useful as a marker for cellular characterization of schwannomas.