During periods of extreme heat individuals with diabetes have greater rates of heat-related morbidity and mortality compared to their non-diabetic counterparts. The reason for this discrepancy is currently unknown. Furthermore, there is a lack of information about whether or not individuals with type 1 diabetes are at a thermoregulatory disadvantage during strenuous physical activity especially when performed in the heat. Purpose: This review discusses the current literature pertaining to thermoregulatory responses in individuals with type 1 diabetes. Methods: We included 14 reviews and 95 original research articles identified by searches of PubMed and Google Scholar and deemed relevant to our subject by three independent readers. Results: Individuals with poorly controlled type 1 diabetes may have impaired heat sensation, and a reduced capacity to dissipate heat due to lower skin blood flow and sweating responses and a greater tendency towards dehydration compared to individuals without diabetes. Impairments may be attenuated or absent in those with good blood glucose control. We found no published studies examining thermoregulatory responses to physical activity in the heat in individuals with type 1 diabetes. Conclusions: Type 1 diabetes may cause impairments in heat loss resulting in a greater level of thermal strain. Advancement in our understanding about the effects of type 1 diabetes on the heat stress response, especially during different challenges to human heat balance associated with changes in both environmental heat load and metabolic heat production (physical activity), will help us to determine where the risk of heat-illness/injury actually exists.