Testicular hamartomas (TH) is a benign condition. An association of TH with Cowden disease (CD) is known. Ultrasound features of hamartomas are often diagnostic. We present a case of Cowden disease with TH and an epididymal tumor. Imaging features of TH and its differentials has been discussed. Although, association of Cowden disease with many malignancies have been documented, epididymal tumor has not been described. To our knowledge, this paper is the first to describe epididymal tumor in association with Cowden disease.