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BACKGROUND: Abdominal cutaneous nerve entrapment syndrome (ACNES) is a frequently overlooked cause of chronic abdominal pain. We aim to outline the current available literature concerning the treatment of patients diagnosed with ACNES. DATA SOURCES: A systematic search in PubMed, EMBASE, CINAHL, and Cochrane databases was performed. Seven studies were included; describing trigger point injection (TPI) or anterior neurectomy as stand-alone procedure, TPI followed by anterior neurectomy as stepwise regimen, and nerve stimulation and phenolization. After TPI, 86% of the patients showed successful response, 76% at long-term follow-up. Two other studies report successful treatment in 50% of patients. In the included trial using anterior neurectomy, 73% vs 18% of the patients demonstrate a successful pain response in the neurectomy and sham group, respectively. Two cohort studies showed that 69% and 61% of the neurectomy group reported to be satisfied at 18 months and 32 months follow-up, respectively. CONCLUSIONS: There is significant pain relief after injections and anterior neurectomy. Awareness of the diagnosis is important. The validity of currently used diagnostic criteria needs to be evaluated in additional studies.