OBJECTIVE Case report of a mature cystic teratoma (dermoid cyst), pseudopilomatrixoma like variant. METHODS 53-year old patient with a left testicular mass, diagnosed as mature cystic teratoma of the testis(dermoid cyst), the pilomatrixoma-like variant, after radical orchiectomy. Tumoral markers were negative and no extension was observed. The patient is disease-free after 6 months follow-up. RESULTS Testicular teratoma is a tumour composed of cells derived from the three embryonic layers (ectoderm, endoderm and mesoderm). They can appear at any age, but they are much more prevalent in childhood. They appear at any age, but are much more common in childhood, where they comprise up to 30% of all tumours. They are much less prevalent in adults,representing only 7% of all testicular germ cell tumours CONCLUSIONS Prepubertal cases are invariably benign in nature, regardless of their histology. However, cases which appear after puberty are potentially malignant, even if histologically pure. Mature cystic teratoma (dermoid cyst) is the only exception to this rule, as no malignant degeneration of these types of tumours has been published.