Background and Importance: Third nerve palsies in the pediatric population are most commonly caused by trauma, tumors, or vascular abnormalities. Cystic oculomotor nerve neuropathies, however, are rare. We report the case of a symptomatic cyst along and within the oculomotor nerve, which has not been described previously. Clinical Presentation: Here, we report a case of a 3-yr-old girl presenting with a progressive painless oculomotor nerve palsy. A magnetic resonance imaging revealed a cystic formation along the cisternal and cavernous course of the nerve. Due to lack of alternative treatment options, surgery was offered. Intraoperative direct nerve stimulation allowed for identification of a non-functional part of the cyst wall and open fenestration and biopsy were executed. Histopathology revealed neuritis. Serology was negative for various pathogens. The oculomotor palsy rapidly resolved. At a follow-up 5 yr after surgery, the girl is asymptomatic and the cisternal part of the cyst remains collapsed. Conclusion: This is the first report of a symptomatic cyst along and within the oculomotor nerve treated effectively with open fenestration and decompression highlighting the importance of intraoperative neuromonitoring in cranial nerve surgery. Uncertainty remains regarding the etiology of this disease.