Objectives Through this review, we sought to accomplish two goals: 1) analyze and synthesize the published case reports on upper extremity necrotizing fasciitis and 2) identify any trends in the diagnosis and treatment of upper extremity necrotizing fasciitis. Methods Studies included in this systematic review were conducted per PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines with the following eligibility criteria: (1) single case or limited case series, (2) established diagnosis of necrotizing fasciitis in the hand, wrist, or forearm, (3) English language publication. Results Most of the patients in this review (38, (70.37%)) presented with an inciting injury. A minority of patients had other associated co-morbidities independent of injury, including Diabetes Mellitus, immunosuppression, and IV drug use. The most common infectious agents were gram-positive cocci, identified in 32 (59.26%) cases, followed by gram-negative bacilli in 21 (38.89%) cases. The most common pathogen was Group A streptococcus (GAS), reported in 15 (27.78%) cases, followed by Vibrio vulnificus in 6 (11.11%) cases. Most patients were managed with empiric antibiotic treatment. Fifty patients (92.6%) reported surgical intervention and 45 (83.3%) had at least one debridement. Most patients in the cases reviewed had either no or minimal long-term sequelae following their infection. Conclusion Early recognition of the signs and symptoms of necrotizing fasciitis is imperative to avoid loss of limb and/or death. Further, aggressive management with empiric antibiotic coverage and rapid surgical intervention improves outcomes in patients with necrotizing fasciitis of the upper extremity.