Macular corneal dystrophy is a rare, hereditary disease. 2 cases of macular corneal dystrophy, receiving penetrating keratoplasty are presented. Both of them showed bilateral involvement of the cornea, onset at puberty, progressive in course and by their early 3rd decade no useful vision remained case 1 revealed no contributory family history, case 2 had a younger brother suffered from the same disease; the corneal lesions of these 2 cases are similar, both showed scattered opacified patches over the axial cornea, the background corneas are ground-glass like in appearance, and the peripheral cornea are rather clear. The pathological changes of these 2 cases showed irregularity of the corneal epithelium and Bowman's membrane, some abnormal material and histiocytes accumulation at the superficial stroma were found; the descemet's membrane and endothelium remained intact. Using special stains, periodic acid Schiff, Masson's trichrome, alcian blue stains, especially the latter, confirmed the diagnosis of macular corneal' dystrophy.