In this study, we report on a case of transdermal paraquat poisoning combined with Pemphigus Vulgaris (PV). The patient was a 68 year old male farmer. He was exposed to paraquat (50% concentration) in a closed environment for 3 h, when the skin and respiratory symptoms of paraquat poisoning occurred. He was admitted to the hospital and diagnosed with transdermal paraquat poisoning combined with PV. After symptomatic treatment using glucocorticoids, immunosuppressive agents, and antibiotics for more than 3 weeks, PV was controlled, and the patient was discharged. Exploring paraquat as an agent in the etiology or induction of PV and its pathogenesis are worthy of further investigation.