Infection is a common complication in patients carrying a central venous catheter (CVC) and is associated with increased morbidity and mortality. Leclercia adecarboxylata is an unusual but emerging pathogen in healthy and immunocompromised patients. We report a case of L. adecarboxylata bacteraemia in a patient with a haemodialysis tunnelled CVC. In accordance with the susceptibility to the tested antimicrobials, a long-course treatment with intravenous gentamicin plus amoxicillin-clavulanic acid and gentamicin-lock therapy was adopted. The patient had a full recovery and the catheter was not removed. We also performed a systematic PubMed/Medline and Scopus review of peer-reviewed English papers on L. adecarboxylata infections, focusing on bacteraemia in patients with different types of CVCs. Moreover, we suggest a treatment algorithm to preserve the patient and maintain the CVC.