We report an unusual case of a malignant granular cell tumor of the left lumbar region in 63-year old woman-diagnosed, consulted and treated with surgical resection (R1) and radiotherapy, followed up for 2 years with lung metastases after 22 months. Furthermore, we discuss histopathological differential diagnosis and current criteria for malignancy, as well as available options for systemic treatment in view of cytogenetic and molecular genetic characteristics of the tumor.