Immunoglobulin A (IgA) nephropathy is the most common primary glomerulonephritis worldwide. The pathogenesis is still unknown and treatment has not yet been established. Rarely it can be associated with other disorders. Its association with hereditary lymphoedema is not reported before. We report four patients, a 60-year-old father, his two sons and his daughter, with hereditary lymphoedema. The family had nine members and in four of them lymphoedema was evident. The other members had neither lymphoedema nor IgA nephropathy. This is the first report of IgA nephropathy in association with hereditary lymphoedema.