Telemedical technologies provide significant benefits in sports for performance monitoring and early recognition of many medical issues, especially when sports are practised outside a regulated playing field, where participants are exposed to rapidly changing environmental conditions or specialised medical assistance is unavailable. We provide a review of the medical literature on the use of telemedicine in adventure and extreme sports. Out of 2715 unique sport citations from 4 scientific databases 16 papers met the criteria, which included all research papers exploring the use of telemedicine for monitoring performance and health status in extreme environments. Their quality was assessed by a double-anonymised review with a specifically designed four-item scoring system. Telemedicine was used in high-mountain sports (37.5%; n = 6), winter sports (18.7%; n = 3), water sports (25%; n = 4), and long-distance land sports (18.7%; n = 3). Telemedicine was used for data transfer, teleconsulting, and the execution of remote-controlled procedures, including imaging diagnostics. Telemedical technologies were also used to diagnose and treat sport-related and environmentally impacted injuries, including emergencies in three extreme conditions: high mountains, ultraendurance activities, and in/under the water. By highlighting sport-specific movement patterns or physiological and pathological responses in extreme climatic conditions and environments, telemedicine may result in better preparation and development of strategies for an in-depth understanding of the stress of the metabolic, cardiorespiratory, biomechanical, or neuromuscular system, potentially resulting in performance improvement and injury prevention.