Lesions, which commonly grow and protrude beneath the distal end of a toe nail and present to the podiatrist or foot and ankle surgeon, include subungual exostosis or enchondroma and a Nora lesion. Neurothekeoma is a benign dermal tumour of the peripheral nerve sheath that usually affects the skin of the head, neck, shoulders and arms. It occurs less commonly in the lower limbs and has only been reported twice in a subungual location. A case of subungual neurothekeoma that recurred twice due to inadequate margins of resection is presented.

Although rare, neurothekeoma should be included in the differential diagnosis of a subungual lesion. Histopathological diagnosis is reached by differential immunostaining. Adequate clear margins of resection are recommended to prevent recurrence.

Neurothekeoma is a rare myxoma of the peripheral nerve sheath. The current report presents a case of a giant neurothekeoma with a partially-formed capsule, scapula erosion and unclear biological behavior, which originated in the intermuscular space between the left trapezius muscle and scapula. The patient was initially misdiagnosed with a fibromatosis using computed tomography and magnetic resonance image scanning. Diagnosis of the neoplasm was confirmed by pathological and immunohistochemical examination, revealing a neurothekeoma with unclear biological behavior. The patient underwent a wide and complete local resection. Using a comprehensive postoperative follow-up strategy, it was determined that the patient recovered well. The tumor was ~17×16×10 cm in size and was in contact with the scapula. The purpose of the present study was to describe a rare giant neurothekeoma and review the diagnostic techniques utilized to reach a definitive diagnosis. Histopathological and immunohistochemical analyses were recommended for the diagnosis of neurothekeoma. There have been no previous reports regarding neurothekeomas exhibiting malignant transformation. Early and complete surgical resection is considered to be an effective method of treating this type of neurothekeoma.