Liponeurocytoma is a rare benign tumor of the central nervous system, which develops mainly in adult patients within the posterior fossa. The WHO has categorized this entity in their last classification of 2016 as a benign grade II tumor. Histopathological characteristics contain neuronal and variable astrocytic differentiation with foci of lipomatous distinction. Up to now only a few case reports and case series are published and the knowledge of this tumor is still very limited. General treatment guidelines do not exist. Aim of this study was to analyze the literature to create treatment guidelines. PRISMA (i.e., Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines were followed to query existing online databases between January 1, 1978 and May 15, 2018. Systematic review of the literature revealed 73 cases (40 female, 33 male) of a liponeurocytoma whereof 59 cases (80.8%) occurred in the posterior fossa. Ki-67/MIB-1 proliferation index was described in 58 cases showing a mean value of 3.73% ± 4.01. Follow-up was performed in 49 cases with a median length of 52.02 ± 50.52 months, showing tumor recurrence in 14 cases (28.57%). Tumor recurrence was observed in only one case (1/12, 8.33%) after adjuvant radiotherapy, while tumor recurrence was seen in 13/29 patients (44.83%), who did not receive adjuvant radiotherapy. Liponeurocytomas are rare benign tumors, occurring mainly in the cerebellum. Therapy of choice is surgery. Postoperative radiotherapy seems to lower the risk of tumor recurrence and should be offered to the patient.