Neurenteric cysts (NCs) are rare congenital lesions that are thought to result from the persistence of the neurenteric canal connecting primitive gut and neural tube. Despite the congenital nature, NCs can be diagnosed at any age and at a similar frequency in women and men. To our knowledge, 140 intracranial NCs, confirmed by histology, including the patient presented in this review, have been reported since 1952. Parenchymal NCs are extremely rare, and there are no publications describing the intra-axial NCs of the brainstem at the moment.A 19-year-old female presented to the clinic with the following complaints: moderate dysphagia (two to three times per day) for and liquids and solids, dysphonia, vertigo, spontaneous nystagmus, imbalance, and numbness in the left side of the body, including the face. The magnetic resonance imaging (MRI) of the brain showed a well-defined lesion centered in the medulla. The patient underwent a small right-sided keyhole retro-sigmoid craniotomy. Just under the sulcus, a cyst containing pathological amorphous gray-yellow liquid was evacuated. Accessible parts of the capsule were resected without brain injury. Residual particles of the capsule were coagulated.Two months after the operation, the patient presented with similar symptoms. We used the previous craniotomy during the second surgery. After the evacuation of the cyst, a silicone stent was set for connecting with the cerebellopontine cistern and preventing new synechiae formation. As surely as after the first surgery, all neurological symptoms gradually regressed. In two months after surgery, deglutition and sensations recovered, and hemiparesis and imbalance decreased. Postoperative MRI examination two months after surgery showed no evidence of cyst recurrence.Intra-axial NCs are a rare group of congenital pathological lesions with a favorable prognosis. There are no publications of brainstem NCs with intra-axial localization to date. The treatment of choice in this group of patients is complete microsurgical excision followed by long-term observation.