Nasal septum perforation is an uncommon and not well known feature of lupus erythematosus (LE). In general, it occurs during exacerbations and in a context of systemic vasculitis. Very rarely it can be a presenting sign, accompanying more usual manifestations of LE. We report the case of a 30-year-old woman who presented with a 2-year history of painful, slowly progressive nasal septum perforation. Laboratory study disclosed positive antinuclear antibodies, circulating immune complexes, hypocomplementemia, nuclear epidermal deposition of IgG in normal skin and transitory positive antiphospholipid antibodies. Symmetric peripheral joint arthritis, photosensitivity and diffuse alopecia subsequently developed. This case seems unique in that the nasal septum perforation occurred as an isolated presenting sign; it emphasizes the value of this feature in the diagnosis of LE.