ObjectivesThis article represents the first report, to our knowledge, of a primary retroperitoneal neurofibroma that mimics a perineal hernia. In addition to the case report, a review of pelvic floor hernias and retroperitoneal hernias is presentedMethods and MaterialsThe information for the case report was obtained from hospital records and the office records of two of the authors (Thad R. Denehy, MD and James L. Breen, MD).DiscussionRetroperitoneal tumors and pelvic floor hernias are rare clinical entities. Care should be undertaken not to prematurely diagnose either of these conditions; this will lead to unnecessary delay in intervention, inadequate preoperative evaluation and suboptimal outcome.ConclusionThis article emphasizes the importance of operative management in the treatment of retroperitoneal tumors. A careful preoperative workup, and an experienced surgeon are imperative. It is crucial that the management of each case is tailored to the resources available.