Systemic sclerosis can occur in overlap with other connective tissue diseases. However, the association of systemic sclerosis and lupus features with systemic lymphadenopathy is distinctly uncommon. A 34-year-old Korean woman with typical diffuse systemic sclerosis and overlapping features of systemic lupus erythematosus (elebvaed anti-dsDNA, 45.11 IU/mL) presented with systemic lymphadenopathy. She reported pain accompanied by lymphadenopathy, especially in the inguinal, lower neck, and axillar areas. Biopsy of the right supraclavicular lymph node showed reactive hyperplasia, a perifollicular pattern, and perinodal fibrosis. After the exclusion of other causable disease like tumor, infection or activated lupus, we considered it as reactive change to the disease activity of systemic sclerosis. Following treatment with deflazacort, partial remission was achieved with pain relief and resolved lymphadenopathy without renal complication. Systemic lymphadenopathy is not a well-described feature of systemic sclerosis. We report a case of typical diffuse systemic sclerosis with overlapping lupus features accompanied by systemic lymphadenopathy.