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Frey syndrome has been occasionally reported to occur in subjects without history of surgery or diabetes but this variant of Frey syndrome has not been systematically investigated. We searched for original articles of Frey syndrome unrelated to surgery or diabetes without date and language restriction. Article selection and data extraction were performed in duplicate. Our systematic review included 76 reports describing 121 individual cases (67 males and 54 females) of Frey syndrome not associated with surgery or diabetes. The age at onset of symptoms was\u2009\u2264\u200918\u00a0years in 113 (93%) cases. The time to diagnosis was 12\u00a0months or more in 55 (45%) cases. On the other hand, an allergy evaluation was performed in half of the cases. A possible cause for Frey syndrome was detected in 85 (70%) cases, most frequently history of forceps birth (<jats:italic>N<\/jats:italic>\u2009=\u200963; 52%). The majority of the remaining 22 cases occurred after a blunt face trauma, following an auriculotemporal nerve neuritis or in association with a neurocutaneous syndrome. The cause underlying Frey syndrome was unknown in 36 cases.<\/jats:p><jats:p><jats:italic>\u00a0 \u00a0Conclusion<\/jats:italic>: Frey syndrome not associated with surgery or diabetes almost exclusively affects subjects in pediatric age and is uncommon and underrecognized. Most cases occur after forceps birth. There is a need to expand awareness of this pseudo-allergic reaction among pediatricians and allergists.<jats:table-wrap><jats:table><jats:tbody><jats:tr><jats:td colspan=\"2\"><jats:bold>What is Known:<\/jats:bold><jats:italic>\u2022 Pre-auricular reddening, sweating, and warmth in response to mastication or a salivary stimulus characterize Frey syndrome.<\/jats:italic><jats:italic>\u2022 It usually occurs after salivary gland surgery and in diabetes.<\/jats:italic><\/jats:td><\/jats:tr><jats:tr><jats:td colspan=\"2\"><jats:bold>What is New:<\/jats:bold><jats:italic>\u2022 In children, Frey syndrome is rare, and most cases occur after a forceps-assisted birth.<\/jats:italic><jats:italic>\u2022 In childhood, this condition is often erroneously attributed to food allergy.<\/jats:italic><\/jats:td><\/jats:tr><\/jats:tbody><\/jats:table><\/jats:table-wrap><\/jats:p>","DOI":"10.1007\/s00431-022-04415-w","type":"journal-article","created":{"date-parts":[[2022,2,19]],"date-time":"2022-02-19T08:09:52Z","timestamp":1645258192000},"page":"2127-2134","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Auriculotemporal Frey syndrome not associated with surgery or diabetes: systematic review"],"prefix":"10.1007","volume":"181","author":[{"given":"C\u00e9line","family":"Betti","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3829-4254","authenticated-orcid":false,"given":"Gregorio P.","family":"Milani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastiano A. 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