Tumors arising in the spermatic cord are uncommon and form a heterogeneous group of neoplasms. A variety of both benign and malignant lesions have been demonstrated. Benign tumors represent 80% of the total, but unfortunately clinical and sonographic features are not specific and they cannot safely differentiate a teratomatous or a malignant neoplasm from benign tumors. We report a case of a huge epidermoid cyst of the spermatic cord in an adult patient confirmed after local excision. The etiology, differential diagnosis, and management alternatives are discussed.