Abstract
The granular cell tumor or Abrikossoff's tumor is a rare tumor, most often benign in evolution. Malignant forms are exceptional. We report, here, a very rare case of granular cell tumor, localized in the perianal region, in a 54-year-old woman with lung metastases. CT and MRI with contrast showed a locally advanced tumor process in the right para-anal region associated with multiple "balloon release" lung lesions. The diagnosis was confirmed by immunostaining after surgical biopsy. Very few cases of malignant granular cell tumors with lung metastasis have been reported in the literature.