AbstractPityriasis rubra pilaris (PRP) is a rare, chronic cutaneous inflammatory disorder of keratinization affecting adults, children and patients with HIV. The pathogenesis of PRP is not fully understood with clinical presentations, and severity remains highly variable. Current treatment modalities for PRP result in recalcitrant disease with potentially unfavourable therapeutic side effects and low tolerability. Due to the rarity of this condition, limited data on treatment efficacies and established management guidelines are lacking. The psychological burden of PRP is detrimental to the quality of life of patients affected with PRP and remains a persistent gap of knowledge. Here, we provide a review of the literature, summarizing new developments in the treatment of PRP and a case report of a patient treated successfully with the anti‐interleukin (IL)‐23p19 antibody Risankizumab with sustained clinical improvement. Risankizumab appears to be an effective and safe treatment for PRP in Asian‐Arabic patients. Further studies are required to assess the efficacy, safety and tolerability of newer targeted therapies for severe PRP.