Neurothekeomas are benign tumours of nerve sheath origin. Neurothekeomas present most commonly on the head and neck and upper extremities. These are usually small, slow growing tumours. Most patients with neurothekeoma are young women. We describe a 1-year-old infant with a large and extensive neurothekeoma in the upper extremity. We believe that our patient is one of the youngest cases and the tumour is probably the largest and most extensive neurothekeoma reported in the literature.