Protein losing enteropathy (PLE) due to systemic lupus erythematosus (SLE) is relatively uncommon. PLE may be appeared sequentially after the diagnosis of SLE or concurrently with SLE. In most of concurrent cases, PLE was diagnosed one of various symptoms of SLE. Cases of PLE as the initial and only clinical presentation of SLE have been rarely reported. We described a 30-year old woman with general edema and abdominal distension was diagnosed PLE after stool alpha 1 antitrypsin clearance test. Her symptoms were getting worse even though the treatment with intravenous albumin. She was finally diagnosed PLE associated with SLE by additional laboratory findings (positive antinuclear antibody and anti-dsDNA IgG and low C3, C4 and CH50). She was treated with high dose of steroids and her symptoms were improved.