Background Necrotising fasciitis is a rapidly progressing soft-tissue infection with a low incidence that carries a relevant risk of morbidity and mortality. Although necrotising fasciitis is often fatal in adults, its case fatality rate seems to be lower in children. A highly variable clinical presentation makes the diagnosis challenging, which often results in misdiagnosis and time-delay to therapy. Methods We conducted a protocol-based systematic review to identify specific features of necrotising fasciitis in children aged one month to 17 years. We searched ’PubMed’, ’Web of Science’ and ’SCOPUS’ for relevant literature. Primary outcomes were incidence and case fatality rates in population-based studies, and skin symptoms on presentation. We also assessed signs of systemic illness, causative organisms, predisposing factors, and reconstructive procedures as secondary outcomes. Results We included five studies reporting incidence and case fatality rates, two case-control studies, and 298 cases from 195 reports. Incidence rates varied between 0.022 and 0.843 per 100,000 children per year with a case-fatality rate ranging from 0% to 14.3%. The most frequent skin symptoms were erythema (58.7%; 175/298) and swelling (48%; 143/298), whereas all other symptoms occurred in less than 50% of cases. The majority of cases had fever (76.7%; 188/245), but other signs of systemic illness were present in less than half of the cohort. Group-A streptococci accounted for 44.8% (132/298) followed by Gram-negative rods in 29.8% (88/295), while polymicrobial infections occurred in 17.3% (51/295). Extremities were affected in 45.6% (136/298), of which 73.5% (100/136) occurred in the lower extremities. Skin grafts were necessary in 51.6% (84/162) of the pooled cases, while flaps were seldom used (10.5%; 17/162). The vast majority of included reports originate from developed countries. Conclusions Clinical suspicion remains the key to diagnose necrotising fasciitis. A combination of swelling, pain, erythema, and a systemic inflammatory response syndrome might indicate necrotising fasciitis. Incidence and case-fatality rates in children are much smaller than in adults, although there seems to be a relevant risk of morbidity indicated by the high percentage of skin grafts. Systematic multi-institutional research efforts are necessary to improve early diagnosis on necrotising fasciits. Electronic supplementary material The online version of this article (10.1186/s12879-019-3941-3) contains supplementary material, which is available to authorized users.

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