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Pityriasis rubra pilaris (PRP) is generally treated with single or combination regimens of topical agents, systemic retinoids, methotrexate, biologics, various other immunosuppressant agents, and light therapy; however, cases of PRP that are not amenable to these therapies can be challenging to control. We present the case of a male with biopsy-proven juvenile PRP followed for ten years, with failure to respond to all traditional treatment regimens. Significant improvement in the patient's cutaneous and rheumatological symptoms occurred when the patient was treated with bexarotene (Targretin®). To our knowledge, this represents the first reported successful treatment of PRP with bexarotene. Thus, treatment with bexarotene may represent a beneficial option for cases of PRP recalcitrant to traditional treatment modalities.