Background: To date, no study has focused on body composition characteristics and on parameters associated with skeletal muscle damage and renal function in runners participating in a 24-h winter race held under extremely cold environmental conditions (average temperature of -14.3 degrees C). Methods: Anthropometric characteristics, plasma urea (PU), plasma creatinine (Pcr), creatine kinase (CK), plasma volume (PV) and total body water (TBW) were assessed pre- and post-race in 20 finishers (14 men and 6 women). Results: In male runners, body mass (BM) (p = 0.003) and body fat (BF) (p = 0.001) decreased [-1.1 kg (-1.4%) and -1.1 kg (-13.4%), respectively]; skeletal muscle mass (SM) and TBW remained stable (p > 0.05). In female runners, BF decreased (p = 0.036) [-1.3 kg (-7.8%)] while BM, SM and TBW remained stable (p > 0.05). The change (Delta) in BM was not related to Delta BF; however, Delta BM was related to Delta SM [r = 0.58, p = 0.007] and Delta TBW (r = 0.59, p = 0.007). Delta SM correlated with Delta TBW (r = 0.51, p = 0.021). Moreover, Delta BF was negatively associated with Delta SM (r = -0.65, p = 0.002). PV (p < 0.001), CK (p < 0.001), Pcr (p = 0.004) and PU (p < 0.001) increased and creatinine clearance (CrCl) decreased (p = 0.002). The decrease in BM was negatively related to the increase in CK (r = -0.71, p < 0.001). Delta Pcr was positively related to Delta PU (r = 0.64, p = 0.002). The decrease in CrCl was negatively associated with the increase in both PU (r = -0.72, p < 0.001) and CK (r = -0.48, p = 0.032). Conclusion: The 24-h running race under extremely cold conditions led to a significant BF decrease, whereas SM and TBW remained stable in both males and females. Nevertheless, the increase in CK, Pcr and PU was related to the damage of SM with transient impaired renal function.