An 18-year-old male patient presented with a swelling over the left post aural region of 3 months duration which gradually increased in size. On examination, the patient appeared well with normal vital signs. Examination of the left ear revealed a 2×2 cm well-defined, solitary, globular, firm, non-tender swelling in the postauricular region with no signs of inflammation. The left pinna, external auditory canal, and tympanic membrane were normal. The patient underwent surgical excision of the swelling, and the specimen was sent for histopathological examination, which revealed a Benign spindle cell tumour. Because the subvariant of benign spindle cell tumour could not be established by histological examination, the specimen was further subjected to immunohistochemistry, which confirmed the tumour as a neurothekeoma of nerve sheath origin. Benign spindle cell tumours, Nerve sheath tumours, Neurothekeomas are very rare superficial soft tissue tumours in adolescent males, hence we are reporting this case and reviewing the literature on these benign spindle cell lesions.
