Purpose of Review <p>Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease that is characterized by recurrent abscesses, tunnels, and nodules. HS requires complex medical management and a multi-faceted treatment approach, particularly in patients where HS coincides with underlying follicular-occlusive or autoinflammatory syndromes. This review aims to synthesize the current literature regarding the treatment of syndromic HS.</p> Recent Findings <p>Recent reports cover a wide range of treatment modalities for syndromic HS. Treatments most frequently utilized included topical and oral antibiotics, retinoids, biologics, corticosteroids, and surgery. Determining clinical efficacy in treatments was limited due to the rare nature of syndromic HS.</p> Summary <p>This review establishes the first comprehensive overview of efficacious treatments in the clinical management of syndromic HS. Understanding the two pathways of syndromic HS and comparing past treatment results can help clinicians in deciding how to manage the treatment of future HS patients. However, additional research is required to discover more efficacious treatment options. </p>