Background: This study reviews the published literature related to nasopharyngeal plasmacytoma. Clinical presentation, demographics, treatment, and outcomes of this uncommon disease have been reported. Methods: A systematic review of studies on nasopharyngeal plasmacytoma from 1935 to 2016 was conducted. A PubMed database search was performed for articles related to this condition along with the bibliographies of those selected articles. Articles were examined for patient data that reported the disease outcome. Results: Fifty-eight journal articles were included in this analysis, comprising of a total of 114 studies indicative of a predilection for cases particularly in men with a mean age of 55 years (sixth decade of life) characterized with symptoms such as nasal obstruction, epistaxis and neck lymphadenopathy. Radiotherapy was the most common treatment modality, followed by a combination of surgery and radiotherapy. Most of the patients were alive with no evidence of the disease after the median follow-up of 59 months. Of the 3 most common treatment modalities, a combination of radiotherapy and surgery had the most favorable outcome for a majority of patients. The existing condition for 8 patients (7%) deteriorated leading to the development of multiple myeloma. Conclusion: This review contains a large pool of information about nasopharyngeal plasmacytoma patients examined to date suggesting that aggressive radiotherapy is the most common treatment modality for this condition. Of the 3 most common treatment modalities, a combination of surgery and radiotherapy was shown to have the best survival outcomes.