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Introduction: Atypical haemolytic uraemic syndrome (aHUS) is a rare, life-threatening disease caused by excessive activation of part of the immune system called complement. Eculizumab is an effective treatment, controlling aHUS in 90% of patients. Due to the risk of relapse, lifelong treatment is currently recommended. Eculizumab treatment is not without problems, foremost being the risk of severe meningococcal infection, the burden of biweekly intravenous injections and the high cost.This paper describes the design of the Stopping Eculizumab Treatment Safely in aHUS trial that aims to establish whether a safety monitoring protocol, including the reintroduction of eculizumab for those who relapse, could be a safe, alternative treatment strategy for patients with aHUS., Methods and Analysis: This is a multicentre, non-randomised, open-label study of eculizumab withdrawal with continuous monitoring of thrombotic microangiopathy-related serious adverse events using the Bayes factor single-arm design. 30 patients will be recruited to withdraw from eculizumab and have regular blood and urine tests for 24 months, to monitor for disease activity. If relapse occurs, treatment will be restarted within 24 hours of presentation. 20 patients will remain on treatment and complete health economic questionnaires only. An embedded qualitative study will explore the views of participants., Ethics and Dissemination: A favourable ethical opinion and approval was obtained from the North East-Tyne & Wear South Research Ethics Committee. Outcomes will be disseminated via peer-reviewed articles and conference presentations., Trial Registration Number: EudraCT number: 2017-003916-37 and ISRCTN number: ISRCTN17503205., Competing Interests: Competing interests: SD has received honoraria for sitting on advisory boards for Alexion and Novartis. DK is a director of and scientific advisor to Gyroscope Therapeutics. DK received advisory board payments from Idorsia, Novartis, ChemoCentryx, Alexion, Apellis, Biomarin and Sarepta. DK’s spouse works for GSK. MM has received honoraria for educational talks and honorarium for national lead of aHUS registry, both from Alexion and travel expenses from Alexion. EKSW has received honoraria for lectures and/or advisory boards for Alexion Pharmaceutical, Biocryst and Novartis. LW has received expenses, honoraria and fees for advisory board participation and talks from Alexion and Roche. NS has given lectures or sat on advisory boards for Alexion Pharmaceutical, Roche, AstraZeneca and Novartis; no personal honoraria, all payments made to the department., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY. Published by BMJ.)