Masquerade syndromes are classically defined as entities which emulate inflammatory conditions but which are in fact due to a neoplastic process. Careful history and examination in concert with appropriate ancillary investigations and histopathologic evaluation of tissue specimens are required in order to make the correct diagnosis. Many conditions may result in an appearance mimicking an inflammatory condition. The authors review neoplastic conditions which may be considered masquerades. The most common of these is primary intraocular lymphoma or primary central nervous system lymphoma, occurring predominately in older individuals. Diagnostic strategies, therapy, and prognosis are reviewed in detail. Other conditions that can be considered masquerade syndromes are reviewed as well, including lymphomatous and nonlymphomatous conditions, such as melanoma, retinoblastoma, juvenile xanthogranuloma, metastatic lesions, and paraneoplastic syndromes, among others.