Thoracic outlet syndrome (TOS) is a collective term describing a number of symptom complexes attributed to compression of blood vessels and/or nerves at any point between the base of the neck and the axilla. It is frequently considered in the evaluation of patients with acroparesthesias, but one subgroup of TOS, ‘true neurogenic TOS' is a very rare entity. We have seen only one case of true neurogenic thoracic outlet syndrome in the last 5 years, while performing about 500 ENMG examinations per year. In this article we presented our experiences in patients admitted with suspected TOS, and reviewed the literature.