Primary intracranial lymphomas (Weller et al. in Neuro Oncol 14(12):1481-1484, 2012) are an emerging disease and an isolated localization in the pituitary gland i.e. primary pituitary lymphoma (PPL) represents a rare condition. We present an update of the most recent evidence for PPL through a systematic review of the literature. A systematic literature review was conducted using PubMed database up to October 2015. The population was defined as immunocompetent patients with a pathologically confirmed diagnosis of PPL. Patients' characteristics, clinical presentation, radiological features, pathology reports, adjuvant treatment and follow-up data were analyzed. We reported one case of PPL and included our data in this analysis. A total of 33 cases of PPL were identified, including ours. A slight not significant female prevalence was evident, with a mean age of 59 years at diagnosis. Visual troubles and headaches were the most common presenting symptoms. About 80 % of patients presented a cranial nerve (CN) deficit. The most frequently involved were the II and III CN. Anterior hypopituitarism was present in 70 % of cases and a diabetes insipidus in 36 % of cases. PPL was rarely limited to the sella and most often extended to the suprasellar and parasellar space. 70 % of cases underwent resection, 21 % a biopsy. A B-cell lymphoma was isolated in 82 % of cases, a T-cell lymphoma in 15 % and a NK/T cell lymphoma in one case. Overall mean survival rate was 14.4 months (95 % confidence interval 9.0-19.8 months) and there was no difference in terms of survival rates when patients were stratified according to the treatment they received. PPL is an emerging clinical entity. Literature data are too scarce to allow the definition of specific protocols of treatment and the management is based on the guidelines present for PCNSL. The role of surgery aiming at a complete resection of PPL should be reevaluated in wider studies including only this category of patients, to establish the real role of each therapeutic strategy.