Several reports have discussed the neurotoxic effects of star fruit (Averrhoa carambola) in patients with chronic kidney disease (CKD). There is also some evidence highlighting the potentially harmful effects on patients with apparently normal renal function, who after consuming this fruit, developed acute renal injury. We performed a systematic review of the literature in the two main global databases (PubMed and Embase) as well as in Lilacs, for Latin American publications. We also included case reports, case series, or review articles which presented individual patient data. Animal or in vitro studies were excluded. We initially screened 259 references, of which 42 were selected for full-text review and 26 were finally selected for data extraction. A total of 123 patients from eight countries were documented: Brazil, with 47 cases, had the highest reported incidence, followed by Taiwan (36), Bangladesh (20), China and France (8 each), Sri Lanka (2), and Thailand and Colombia (1 each); 28 (22%) of the patients died. Despite the relatively low frequency of star fruit consumption, it has become a global issue. Patients with already diagnosed CKD are the obvious target for preventive measures, but persons with undiagnosed kidney conditions could also be at risk.