Abstract Hidradenitis suppurativa (HS) is a chronic inflammatory condition of the pilosebaceous unit characterized by inflammation and hyperkeratinization. A small but significant proportion of patients with HS have a strong genetic susceptibility to (or a syndromic form of) the disease. Current HS treatment guidelines prioritize patients who manifest classic HS and may therefore not be suitable for the minority of patients harbouring genetically driven forms of disease. In this manuscript, we review the extant literature with regards to therapeutic strategies used for patients with HS having disease-associated genetic variants and syndromic forms of the condition. The findings of this review suggest that patients with HS harbouring underlying genetic variants may not be adequately represented in current European and British HS treatment guidelines. Moreover, these patients may be less responsive to the recommended therapeutic options. We therefore make recommendations for future therapeutic guidelines to incorporate considerations for the management of this patient subset.