Because primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (POAML) is rare, key clinical questions, including the best treatment strategy and the validity of watchful waiting (WW), remain unresolved. Although radiotherapy (RT) is the standard treatment for localized POAML, it is commonly associated with acute and late toxicities. Overall, 128 patients with localized POAML diagnosed and managed at our institution between 1998 and 2015 were retrospectively analyzed. Forty-two patients were initially managed with WW, and 86 received immediate RT. During a median follow-up of 7.2 years (range 0.1–18.4), no patients died of lymphoma progression. Seven patients in the WW group experienced disease progression at the primary site. In the RT group, almost all patients (97.7