BACKGROUND Neurothekeomas are uncommon benign soft tissue tumors of the peripheral nerves. It is commonly found in the upper extremities and in the head and the neck region as a small, asymptomatic, and superficial nodule. METHODS We report a rare case of intraneural neurothekeoma (2.8 χ 2.4 x 1.8 cm) of the median nerve at the wrist in a 56-year-old female, which presented with clinical features of carpal tunnel syndrome. CONCLUSIONS Carpal tunnel syndrome caused by intraneural neurothekeoma has been rarely reported. Clinicians should be aware of this rare cause of carpal tunnel syndrome.