Nodular lymphoid hyperplasia (NLH) of the lung is a benign lymphoproliferative disease, many aspects of which are still unknown. Here we report a rare case of NLH with hemoptysis and multiple lung nodules. A 56-year-old female presented with a complaint of abrupt hemoptysis. The patient had a treatment history of unclassified collagen disease. Chest computed tomography (CT) scan revealed multiple nodules in bilateral lung fields. NLH was confirmed from a biopsy by video-assisted thoracoscopic surgery (VATS). Differentiation from malignant disease, especially mucosa-associated lymphoid tissue (MALT) lymphoma, was required since NLH is rarely associated with hemoptysis and multiple lung nodules. The patient showed no further progression of NLH and thus no treatment was required. This rare case of NLH is here explained and discussed further. The possibility of an association between NLH and collagen disease is also discussed.