•A modified 3-round Delphi survey resulted in a clinician-led guideline in Hirayama disease (HD).•This is the first time to establish a clinician-led guideline for clinical practice in HD.•Given lack of high-grade studies, this experts’ guideline may provide a helpful direction for HD. To establish a clinician-led guideline for the diagnosis and treatment of Hirayama disease (HD) using a modified Delphi technique. Based on a combination of a systematic review and opinion of ten experts, a protocol for the consensus of the diagnosis, treatment and follow-up assessment of HD was established. A modified 3-round Delphi survey was then performed by more than 40 panelists from various countries of the world. Both levels of evidence and levels of agreement were derived in all statements of finial guideline. A total of 47 experts from 6 countries were enrolled in the expert panel in this study. Highly consistent results were achieved during the three Delphi rounds. An expert-led guideline finally constructed includes 24 statements related to diagnosis, treatment and follow-up assessment of HD. The modified Delphi technique used in this study resulted in an expert-led guideline concerning several clinical aspects of HD. This clinician-led guideline may provide a helpful direction for clinical practice with regard to the diagnosis and treatment of HD.