Heritable renal disease was frequently discussed in the medical literature of the past century. Familial nephropathy, described in numerous reports since about 1875, was first confirmed as a clinical syndrome by Guthrie in 1902. And then, Guthrie and others repoted a family with hematuria similar to those already in the literature. In 1927, Alport described a hereditary syndrome of chronic progressive nephropathy and hematuria associated with sensory-neural hearing loss. Main features of the syndrome are considered to be as follows : a definite family history of nephropathy ; hematuria ; proteinuria ; sensory-neural deafness, more common in man and usually not clinically apparent until adolescence ; ocular abnormalities including anterior and posterior lenticonus, microspherophakia, and cataracts; and renal failure which may progress rapidly to death, especially in males. Despite the clinical and pathological descriptions, no one has yet defined the exact significance of Alport's syndrome or explained satisfactory the cause. We recently experienced a case of Alport's syndrome in 37 year old male patient with family history who showed sensory-neural deafness, hematuria, proteinuria without ocular abnormalities. We report this case with review of literatures.