In the previous chapters, a number of noninfectious etiologies of anterior uveitis have been illustrated. In addition, a number of miscellaneous entities may be associated with anterior segment inflammation and present as a diagnostic challenge. The following chapter will describe such cases of noninfectious uveitis which add to the diagnostic conundrum. These cases include rare masquerades such as intraocular mantle cell lymphoma, and Sjögren’s and drug-induced uveitis. In addition, the chapter will also discuss a case of uveitis-glaucoma-hyphema syndrome as well as a case of immune recovery uveitis in a patient with cytomegalovirus retinitis.