We describe a case of epithelioid sarcoma of the median nerve in a 57-year-old woman presenting with symptoms and signs of carpal tunnel syndrome for 2 years. The clinical examination was suggestive of a wrist ganglion compressing the median nerve. Magnetic resonance imaging (MRI) showed a 5 cm x 3 cm mass involving the median nerve in the carpal tunnel and appearances mimicked a benign peripheral nerve sheath tumour. This report illustrates a rare tumour presenting in a rare location and emphasizes the atypical clinical and MRI features that should alert the radiologist to the possibility of a rare sarcoma mimicking a benign peripheral nerve sheath tumour.