Intraneural perineurioma is a rare tumour that affects peripheral nerves and, based on its histological features, may be confused with hereditary motor and sensory neuropathies. Detailed neuropathology, including immunoperoxidase stains and electron microscopy, is vital to distinguish these conditions. We report two patients with intraneural perineurioma that demonstrate salient features of this tumour. The first patient is the longest reported follow-up of an intraneural perineurioma; extension of the lesion was observed over 14years. The second patient is an 11-year-old female whose treatment highlights some of the controversy surrounding the management of these lesions, and the importance of thorough macroscopic and microscopic assessment by pathologists, including the status of surgical resection margins.