Bilateral acute compartment syndrome of the legs is a very rare presentation that requires emergency surgical intervention. Atraumatic bilateral cases are almost unheard of in medicine. There is currently no link between compartment syndrome and cognitive impairment or mental health.A systematic literature search was performed in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines using the following keywords in multiple databases: compartment syndrome, atraumatic, spontaneous, bilateral, both, lower leg, acute, compartmental pressure, and fasciotomy. Atraumatic, bilateral, acute, and confirmed compartment syndrome cases were included.In total, 33 cases of atraumatic bilateral acute compartment syndrome (ABACS) were identified, of those 72.7% of cases were males. A form of cognitive impairment was found in 66% of cases. The medical history of the cases included substance abuse (nine patients), mental health illness (seven patients), and hypothyroidism (four patients). Within the reports, there was evidence of a misdiagnosis or delayed management in 19 cases (57.6%). Creatinine kinase (CK) was measured in 28 cases with a mean CK of 110,893 IU/L. Compartment pressure measurements were used in only 12 cases. A total of 29 cases were managed with bilateral four-compartment fasciotomy. This review highlights that ABACS is a condition with high rates of misdiagnosis or delay in treatment. Associations found included patients with cognitive impairment on presentation, mental health conditions, substance misuse, and elevated levels of CK. In addition, this review demonstrates that this condition is less rare than previously thought with serious morbidity and mortality.