Background and objectives: Ultra-trail races can cause episodes of acute kidney injury (AKI) and exercise-associated hyponatremia (EAH) in healthy subjects without previous renal pathology. This systematic review aims to review the incidence of these two syndromes together and separately taking into account the length and elevation of the ultra-trail race examined. Materials and Methods: A systematic review was conducted through electronic search in four electronic databases (PubMed, EBSCO, Web of Science and Alcorze). Results: A total of 1127 articles published between January 2006 and December 31, 2021 were included, 28 of which met the inclusion criteria. The studies were categorized according to the length and stages of the race in four categories: medium (42 to 69 km), long (70 to 99 km), extra (>100 km) and multi-stage if they included various stages. A total of 2950 runners (666 females and 2284 males) were extracted from 28 publications. The AKI incidence found was 42.04% (468 cases of 1113), and 195 of 2065 were diagnosed with EAH, accounting for 9.11%. The concurrence of both pathologies together reached 11.84% (27 individuals) from a total of 228 runners with AKI and EAH simultaneously analyzed. Sorted by race category, the AKI+EAH cases were distributed as follows: 18 of 27 in the extra (13.63% and n = 132), 4 in the large (5.79% and n = 69) and 5 in the medium category (18.15% and n = 27). Conclusions: According to these results, extra and medium races showed a similar incidence of AKI+EAH. These findings underline the importance of the duration and intensity of the race and may make them responsible for the etiology of these medical conditions. Due to their variable incidence, EAH and AKI are often underdiagnosed, leading to poorer prognosis, increased condition seriousness and hindered treatment. The results of this review urge participants, coaches and race organizers to take measures to improve the early diagnosis and urgent treatment of possible EAH and AKI cases.