Introduction and clinical case: We report a new unusual case of a patient without known familiar history of melanocytic schwannoma or Carney complex with multiple melanocytic schwannoma in the spinal canal. Histologically, the tumor was composed of irregular interlacing spindle-shaped cells with pigments. The diagnosis is made by immunohistochemical techniques and electron microscopy findings.
Conclusion: We review the literature, and the different theories on the origin of these tumors are discussed.