Testicular microlithiasis is a rare condition that has characteristic sonographic and histologic features. It is often associated with premalignant changes and malignant neoplasms of the testes. We report a case of testicular microlithiasis associated with teratocarcinoma and intratubular germ cell neoplasia. Three previously reported cases documented development of malignant germ cell tumor during the clinical follow-up period of patients with testicular microlithiasis. We think that testicular microlithiasis is strongly associated with testicular neoplasms, and that coexistence of intratubular germ cell neoplasia and malignant germ cell tumors with testicular microlithiasis is common.