We report a 40-year-old female patient presenting with isolated left spinal accessory neuropathy that developed insidiously over 6 years. She complained of ill-defined deep neck and shoulder pain. On examination, prominent sternocleidomastoid and trapezoid muscle weakness and atrophy, shoulder instability, and lateral scapular winging were observed. MRI identified a small mass of the cisternal portion of the spinal accessory nerve. Its appearance was typical of schwannoma. Surgical treatment was not offered because of the small tumor size, lack of mass effect and the questionable functional recovery in the presence of muscular atrophy.Isolated spinal accessory neuropathy (SAN) is not uncommon after iatrogenic surgical injury of the accessory nerve (AN) during various procedures in the posterior triangle of the neck [1,2]. Less frequent aetiologies include traction injury, blunt or penetrating trauma, neuralgic amyotrophy, infection, or tumors involving the AN along its complicated course (intraaxially; intradurally in the high cervical segment of the spinal canal and at the foramen magnum; extradurally but intracranially, at the skull base/jugular foramen; extracranially, distal to the jugular foramen in the neck) [1,2]. Schwannomas of the AN are very rare and occur extracranially [3] or intracranially. The intracranial ones are further divided by location into intrajugular and intracisternal. Intrajugular masses result in jugular foramen syndrome and only occasionally present as isolated SAN [4].Intracisternal AN schwannomas are exceedingly rare and usually manifest by the mass effects they produce [3]. The patient we report is -to the best of our knowledge -the first with this type of neoplasm to present with isolated SAN.A 40-year-old female patient presented with poorly localized deep pain in the neck and left shoulder of 6 years duration. Her complaints were stationary over the last years, and she denied any other symptoms. There was no history of surgery or trauma to the neck. Examination showed prominent atrophy of the left trapezius and sternocleidomastoid (SCM) muscles (Fig. 1), drooping of the left shoulder and lateral scapular winging, accentuated by shoulder abduction.Muscle power testing (MRC scale) was 4/5 for the trapezius and 3/ 5 for the SCM. Abduction of the left arm was limited to 90 degrees. The rest of her neurological examination was normal. In particular, there was no evidence of tongue atrophy or asymmetry, the soft palate and uvula were symmetrical with preserved gag reflexes. Direct laryngoscopy was normal. Formal gustometry was not performed, but the patient denied any changes in taste sensation and testing for sour of the posterior third of the tongue (citric acid strip 16.5%) revealed no loss of taste. Clinical impression was of an isolated left spinal accessory neuropathy (SAN).Nerve conduction studies revealed normal latencies but markedly reduced compound muscle action potential amplitude of the left trapezius muscle (0.4 mV versus 6 mV on the right) (Fig. 2).Needle EMG examination...