<h4>Rationale</h4>Pseudo-pseudo Meigs syndrome (PPMS), characterized by pleural effusion, ascites, and elevated cancer antigen-125 (CA-125) levels, is a rare complication of autoimmune diseases, particularly of systemic lupus erythematosus-systemic sclerosis (SLE-SSc) overlap syndrome.<h4>Patient concerns</h4>A 39-year-old female presented with progressive symptoms, including finger swelling (since 13 years), skin hardening (since 10 years), bilateral second toe ulceration (since 3 months), and abdominal distention (since 1 month). Here, MUC16 is linked to primary progressive multiple sclerosis.