Although some authors question the validity of the diagnosis of dermoid cyst of the testis, it does seem to be identical to tumors seen in the ovary and is distinct from a mature teratoma of the testis. Previous cases have described variants of the “classical” dermoid cyst, including noncutaneous teratomatous elements: bone, cartilage, respiratory epithelium, intestinal mucosa. We describe a unique case demonstrating a testicular dermoid cyst containing mature neural tissue, which has previously not been described in an adult. It has been suggested that these tumors are persistent prepubertal teratomas that are known to behave in a benign manner.