Abstract. Pilocytic astrocytoma (PA) is a rare glioma, which generally occurs in children and young adults. In adult patients, the majority of PA tumors are supratentorial. Due to the low morbidity rate of the disease in adults, PA is frequently misdiagnosed and mistreated. In the present study, this rare disease was successfully treated. The study reported the case of a 48-year-old patient with a cervicomedullary occupying lesion, who complained of numbness and pain of the right limbs that persisted for >10 years, with aggravation for 1 month. A magnetic resonance imaging scan showed a sharp cervicomedullary mass extending from the lower medulla to the cervical vertebra C3 level. Intraoperatively, the medulla and upper cervical cord were found to be well-stacked. Immediately after ingression into the spinal cord along the dorsal median sulcus, the tumor mass was detected and had a gray fish-like appearance, moderate blood supply and clear boundary. After intratumoral decompression, total excision was achieved. Postoperative pathological examination confirmed that the tumor was a PA. Following discharge, the patient did not suffer from any symptoms of the lower cranial nerves and was able to walk with limited assistance.