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Malignant peripheral nerve sheath tumor (MPNST) of oral cavity is an extremely uncommon malignancy. Less than 15 cases have been reported since 1973 though none of them describes a distant metastasis. We present a rare case of MPNST of the tongue who presented with features of hypoglossal nerve palsy. Incisional biopsy showed a malignant spindle cell tumor in the sub-epithelial connective tissue. The tumor cells were immune-positive for S-100. He underwent surgery followed by adjuvant chemo-radiation. Later the disease recurred in the form of isolated pelvic bone metastasis. Palliative chemotherapy was offered to him. With this case report we intend to refer to such unusual presentation and pattern of recurrence in a MPNST of tongue.