Linear IgA bullous dermatosis (LABD) is an acquired autoimmune sub-epidermal vesiculobullous disease characterized by continuous linear IgA deposit on the basement membrane zone, as visualized on direct immunofluorescence microscopy. LABD can affect both adults and children. The disease is very uncommon, with a still unknown incidence in the South American population. All confirmed cases of LABD by histological and immunofluorescence in our hospital were studied. The confirmed cases were three females and two males, aged from 8 to 87 years. Precipitant events associated with LABD were drug consumption (non-steroid inflammatory agents in two cases) and ulcerative colitis (one case). Most of our patients were treated with dapsone, resulting in remission. Our series confirms the heterogeneous clinical features of this uncommon disease in concordance with a larger series of patients reported in the literature.