Neurofibromas are benign tumours arising from the Schwann cells of peripheral nerves. They usually occur on the limbs and rarely present at other sites such as the thyroid gland. Lesions associated with the thyroid are usually benign but should be closely followed up. When the presence of a plexiform neurofibroma in the thyroid gland is confirmed by radiological investigations, total thyroidectomy is the treatment of choice because of the substantial risk of malignant transformation. This case report details a rare case of thyroid plexiform neurofibroma in a young female patient with known Von Recklinghausen disease.