Background: Paraneoplastic syndromes are systemic or organ-related functional tumor-associated changes that arise distant to the tumor.
Methods And Results: We present a rare case of a 63-year-old man with dermatomyositis as a paraneoplastic syndrome developing more than a year before clinical manifestations of tonsillar squamous cell carcinoma (SCC). He subsequently developed stage T1N2bM0 IVA tonsillar SCC.