Paraneoplastic presentations are often the initial presenting symptom of a malignant process. A 15-year-old female presented with a progressively growing, sclerotic lesion of the neck restricting the range of motion. She was found to have bilateral ovarian tumors that proved to be diffuse large B-cell lymphoma (DLBCL). After starting cyclophosphamide, vincristine, and prednisone (COP), she had a rapid and complete resolution of the sclerotic lesion, as well as a favorable response to the neoplastic process. In this report, we present a very rare case of extranodal lymphoma associated with a paraneoplastic skin lesion.