An unusual case is presented in which corneal deposits in mid-to-deep stroma with Doyne's posterior pole drusenosis are the initial signs of an IgA-kappa monoclonal gammopathy. The case has several unique features. Corneal deposits have been described previously with IgG and IgM-kappa light chain gammopathies, but to our knowledge have not been described before with IgA-kappa monoclonal gammopathy. Corneal stromal deposits and associated macular drusen have been described with IgM monoclonal gammopathy, but not previously with IgA monoclonal gammopathy. Bietti described superficial perilimbal corneal deposits with fundus albipunctatus, whereas the present case shows diffuse central deep corneal deposits with macular drusen.