Glandular differentiation in peripheral nerve sheath neoplasms is a rare but well recognised phenomenon, believed to represent the least common of the various forms of heterologous differentiation which may occur in tumours of peripheral nerve. This group of tumours--the 'glandular peripheral nerve sheath neoplasms'--are the subject of this short review. The majority of glandular peripheral nerve sheath neoplasms arise in patients with von Recklinghausen's disease and follow a malignant course, but both benign and sporadic forms have now been described. Neither the presence nor the appearance of the glandular foci are believed to influence patient outcome. Predictions of likely biologic behaviour are best based upon an assessment of the nature of the accompanying spindle cell stroma. Glandular peripheral nerve sheath neoplasms must be distinguished from various tumours of soft tissue which may share similar histological features e.g. synovial sarcoma and the epitheloid nerve sheath tumours. Immunohistochemistry and ultrastructural examination may aid in this regard, and have also contributed to our understanding of the nature and origin of the glandular structures, though questions regarding their histogenic origins remain somewhat controversial.