BACKGROUND: Spontaneous spinal subarachnoid hemorrhage (SAH) is extremely rare and occurs in <1% of all cases of SAH. To the best of our knowledge, only 1 case of spontaneous spinal SAH with later development of a symptomatic spinal arachnoid cyst (SAC) has been described in the literature to date. The objective of the present study was to report the challenging clinical management of SAC based on a literature review. CASE DESCRIPTION: We report the case of a 51-year-old woman with acute onset of back pain, neck pain, and headaches with an angiogram-negative supratentorial SAH. Further magnetic resonance imaging screening of the spine revealed additional subarachnoid blood at the level of the thoracic spine, without evidence of vascular malformations. Several weeks after the hemorrhage, the patient developed progressive numbness in her trunk and lower extremities and weakness in her lower extremities. A follow-up magnetic resonance imaging study revealed a large arachnoid cyst at level T4-T7 with spinal cord compression. The patient underwent left hemilaminectomy at T4-T6 and fenestration of the SAC. In a second surgery, right-sided hemilaminectomy at T7 was performed with complete marsupialization of the larger cyst and placement of an intradural shunt. At the 6-month clinical follow-up examination, she showed improvement of her clinical symptoms. CONCLUSIONS: Treatment of secondary SAC is challenging, and surgery of the SAC with or without placement of a shunt is a possible treatment option.