Periorbital edema is a rare manifestation of systemic lupus erythematosus (SLE). We describe here a patient with bilateral periorbital edema as a presenting symptom of SLE. A 23-year-old woman was admitted for a sudden onset of severe periorbital edema that developed 7 days previously. On the basis of the malar rash, arthritis of the right hand and left wrist, leukopenia, proteinuria and the positive anti-nuclear and anti-dsDNA antibodies, she was diagnosed with SLE. Kidney biopsy revealed lupus nephritis (WHO class IV). After treatment with high dose of steroids, intravenous cyclophosphamide and daily hydroxychloroquine, her symptoms resolved, including the periorbital edema. She was discharged with prescriptions for prednisolone and hydroxychloroquine and she remains well.