Hypersensitivity to mosquito bites (HMB) refers to skin reactions such as bullae and necrosis, which occurs after being bitten by mosquitoes and can present with multiple systemic reactions such as fever, lymphadenopathy, hepatosplenomegaly simultaneously or subsequently. A 48-year-old male patient presented with recurrent erythema, nodules, papules, vesicles and bullae over upper body and bilateral limbs with itching over the sites of mosquito bite for more than 1 year with low-grade fever and superficial lymph nodes enlargement. The patient's symptoms failed to improve from conventional anti allergic treatment although skin biopsy showed changes of HMB reaction. Subsequently, the lymph node was biopsied and was reported to be nodal marginal zone lymphoma (NMZL), and then the patient was eventually diagnosed with nodal marginal zone lymphoma with HMB. To date only one case of nodal marginal zone lymphoma with HMB has been reported from Korea, and this is the first case to be reported in China.