Background: Pure chronic renal failure as a presentation of a conus neoplasm is a rare entity. In addition, few case reports exist describing an intramedullary teratoma among adults.
Case description: We present a unique case of intramedullary mature cystic teratoma, presenting as isolated renal failure.
Conclusions: As distinguished radiological criteria cease to exist regarding intramedullary teratomas, these neoplasms continue to provide pathological surprises. Surgical excision remains the treatment of choice. The literature review indicates good surgical outcome.