OBJECTIVES: To describe a patient withdedifferentiated liposarcoma of the spermatic cord and toperform a bibliographic review.METHODS: We describe the case of a patient with dedi-fferentiated liposarcoma of the spermatic cord, and con-ducted an extensive search of literature in different databa-ses that support the discussion of the case.RESULTS: A 65 years old male presented a right scrotalmass with progressive growth, and underwent excision ofthe mass (measured 34 x 22 x 17 cm and weighed 5786grams). Pathology reported dedifferentiated liposarcoma ofthe spermatic cord. This patient draws attention to the re-occurrence of papillary thyroid cancer.CONCLUSIONS: Paratesticular tumors are rare entities,derived from mesenchymal tissue. Most of them are be-nign, but up to 30% could be of sarcomatoid type. Theirtreatment is based on the extraction of the mass along withradical orchiectomy, but being a very rare disease is notclear whether the benefit of adjuvant chemotherapy andradiotherapy in these patients is beneficial