Discoid lupus erythematosus (DLE) is a chronic autoimmune condition primarily affecting the head and neck, with palmoplantar involvement (ppDLE) being an uncommon presentation. We present a case of a 46-year-old male with ppDLE and rare perineal involvement, confirmed through biopsy and serological findings, including positive RNP and Sm antibodies. This case underscores the importance of recognizing atypical presentations of DLE, which may indicate a higher risk of systemic progression and require tailored management strategies.