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Malignant peripheral nerve sheath tumor (MPNST) is an aggressive tumor which is often associated with neurofibromatosis type-1 (NF-1). This rare tumor is often associated with nerve roots and bundles in the extremities. Occipital MPNST is rare. Giant size occipital scalp MPNST is extremely rare in literature. The size of tumor often becomes difficult to treat. We present one of the most gigantic occipital MPNSTs (14 × 27 × 44 cm) published in the literature. Case Report: A 27-year-old lady who presented with a huge occipital mass and a history suggestive of bilateral pathological fracture of the tibia. At presentation her occipital mass measured 14 × 27 × 44 cm. Cranial computed tomography (CT) showed evidence of calvarial erosion. She had subtotal tumor resection with histology suggestive of MPNST while immunohistochemistry was positive for S-100. She was referred to the oncology unit for chemoradiation. Conclusion: Huge occipital MPNST presents management challenges because treatment is difficult. This giant MPNST has been added to the body of knowledge while the search for the ultimate solution continues.

A giant occipital malignant peripheral nerve sheath tumor in non-neurofibromatosis person: A rare case and review of literature