Xanthogranulomatous diseases of the eyelids and orbit represent a rare group of histiocytic, granulomatous disorders similar in their predominant cell types but diverse in their prognosis – from benign and self-limited to systemically malignant and ultimately fatal. Distinct clinical syndromes involving xanthogranulomatous inflammation have been described, but clinical overlap exists, suggesting that they are part of a continuum. As the name implies, juvenile xanthogranuloma (JXG) affects children. In adults, the four syndromes of xanthogranulomatous inflammation in the eye and adnexa are adult orbital xanthogranuloma (AOX), adult-onset asthma and periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma (NXG), and Erdheim-Chester disease (ECD). Each of these entities is reviewed in detail herein.