Mucosa-associated lymphoid tissue (MALT) lymphoma is an indolent B cell derived non-Hodgkin's lymphoma. It is the main type of uveal lymphoma and is extremely rare. The pathogenesis of ocular MALT lymphoma remains unclear. It is now considered to be associated with many causes. The manifestations of primary uveal MALT lymphoma differ. So sometimes it is necessary to diagnose depending on diversity of auxiliary tests. Ultrasound examination shows typical low and homogeneous internal reflectivity, with blood flow signal. Optical coherence tomography, fundus imaging, fundus angiography, magnetic resonance imaging and positron emission tomography computerized tomography can assist diagnosing. Primary uveal MALT lymphoma is sensitive to radiation therapy, chemotherapy and biotherapy have positive influence too. The prognosis of uveal MALT lymphoma is good, but its early diagnosis is rather challenging. The nonspecific clinical manifestations and the rarity of the disease can confound the initial diagnosis, resulting in delayed treatments which may cause irreversible vision loss.