Chronic exertional compartment syndrome is a debilitating condition primarily associated in highly active individuals with an estimated incidence of approximately 1 in 2000 persons/year. The etiology remains unclear to date. The differential diagnosis includes, but is not limited to stress fractures, medial tibial stress syndrome, and popliteal artery entrapment syndrome. Clinical signs and symptoms include pain in the involved compartment with exertion dissipating quickly after activity. Diagnostic tests include intramuscular compartment pressure testing, magnetic resonance imaging, near-infrared spectrometry as well as shear wave electrography. Treatments consist of nonsurgical, surgical, or the combination of the two. Gait retraining and the use of botulinum toxin appear most promising. Diagnostic lidocaine injections are emerging as a prognostic and mapping tool. Minimal invasive surgical options are being utilized allowing quicker return to activity and decreased morbidity. This article reviews the anatomy, clinical signs and symptoms, diagnostics, nonsurgical, and surgical treatments for chronic exertional compartment syndrome.