Background: High-frequency episodic migraine (HFEM) has gained attention in the field of headache research and clinical practice. In this narrative review, we analyzed the available literature to assess the evidence that could help decide whether HFEM may represent a distinct clinical and/or biological entity within the migraine spectrum., Methods: The output of the literature search included 61 papers that were allocated to one of the following topics: (i) socio-demographic features and burden; (ii) clinical and therapeutic aspects; (iii) pathophysiology; and (iv) classification., Results: Multiple features differentiate subjects with HFEM from low-frequency episodic migraine and from chronic migraine: education, employment rates, quality of life, disability and psychiatric comorbidities load. Some evidence also suggests that HFEM bears a specific profile of activation of cortical and spinal pain-related pathways, possibly related to maladaptive plasticity., Conclusions: Subjects with HFEM bear a distinctive clinical and socio-demographic profile within the episodic migraine group, with a higher disease burden and an increased risk of transitioning to chronic migraine . Recognizing HFEM as a distinct entity is an opportunity for the better understanding of migraine and the spectrum of frequency with which it can manifest, as well as for stimulating further research and more adequate public health approaches., Competing Interests: Declaration of conflicting interestsThe authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article (past 4 years). FC: None. RDI received personal fees for participating in speaking at scientific events from Eli-Lilly, TEVA and Lundbeck. He has participated in an advisory board for Pfizer and AbbVie. MC: None. FB: None. GV received personal fees for participating in speaking at scientific events from Lundbeck. DM reports support from Abbvie for participating in advisory boards; consulting fees from LifeSciences Consultants, fees from Medscape for organizing educational programs; and personal fees from Lundbeck for lecturing at symposia. PP-R reports support for the present study from AbbVie and personal fees for consulting from AbbVie, Eli Lilly, Lundbeck, Medscape, Novartis, Pfizer and Teva. She has received personal fees for speaking from AbbVie, Dr Reddy's, Eli Lilly, Lundbeck, Medscape, Novartis and Teva, and has had grants paid to her research group from AbbVie, AGAUR, EraNet Neuron, FEDER RIS3CAT, Instituto Investigacion Carlos III, International Headache Society, Novartis and Teva. She is a member of the Scientific Advisory Board for Lilly Foundation Spain.PJG reports, over the last 36 months, grants from Celgene and Kallyope; personal fees from Aeon Biopharma, Abbvie, Amgen, eNeura, CoolTech LLC, Dr Reddys’, Eli-Lilly and Company, Epalex, Linpharma, Lundbeck, Man&Science, Novartis, Pfizer, Sanofi, Satsuma, Shiratronics and Teva Pharmaceuticals; personal fees for advice through Gerson Lehrman Group, Guidepoint, SAI Med Partners and Vector Metric; fees for educational materials from CME Outfitters; as well as publishing royalties or fees from Massachusetts Medical Society, Oxford University Press, UptoDate and Wolters Kluwer. CT reports support from Abbvie and Novartis for investigator-initiated trials; consulting fees from Abbvie, Eli Lilly, Dompé, Ipsen, Lundbeck, Pfizer, Teva and Medscape for participating in advisory boards; support from Abbvie, Eli Lilly, Dompé, Ipsen, Lundbeck, Pfizer and Teva for attending meetings; and personal fees from Abbvie, Eli Lilly, Lundbeck, Pfizer and Teva for lecturing at symposia. She is principal investigator of clinical trials sponsored by Abbvie, Biohaven, Eli Lilly, Ipsen, Lundbeck, Pfizer and Teva. She has received research grants from the European Commission, the Italian Ministry of Health and the Migraine Research Foundation.