The objective of this article is to assess the availability and validity of economic evaluations of carotid artery stenosis (CS) diagnosis and treatment. DESIGN: Systematic review of economic evaluations of the diagnosis and treatment of CS. METHODS: Systematic review of full economic evaluations published in Medline and Google Scholar up until 28 February 2012. Based on economic checklists (Evers and Philips), the identified studies were classified as high, medium, or low quality. RESULTS: Twenty-three evaluations were identified. The study quality ranged from 26% to 84% of all achievable points (Evers). Seven studies were of high, eight of medium and eight of low quality. No comparison was made between carotid angioplasty and stenting (CAS) and best medical treatment (BMT). For subjects with severe stenosis, comparisons of carotid endarterectomy (CEA) and BMT were also missing. Three of five studies dealing with pre-operative imaging found that duplex Doppler ultrasound (US) was cost-effective compared with carotid angiogram (AG). CONCLUSIONS: There is a huge lack of high-quality studies and of studies that confirm published results. Also, for a given study quality, the most cost-effective treatment strategy is still unknown in some cases ('CAS' vs. 'BMT', 'US combined with magnetic resonance angiography supplemented with AG' vs. 'US combined with computer tomography angiography').