Gout with systemic lupus erythematosus (SLE) or progressive systemic sclerosis (PSS) has rarely been reported, whereas mixed connective tissue disease (MCTD) with the demonstration of intraarticular monosodium urate crystals has never been reported. We describe an unusual case of MCTD (SLE-PSS) in a 37-year-old woman who developed acute gouty arthritis. Arthrocentesis and synovianalysis may be necessary to differentiate gout from the arthropathy of MCTD.