Pulmonary nodular lymphoid hyperplasia (NLH) is a term first suggested by Kradin and Mark to describe one or more pulmonary nodules or localized lung infiltrates consisting of reactive lymphoid proliferation. To date, there have been only a few cases of pulmonary NLH reported associated with autoimmune disorders. There is no case of NLH associated with Sjogren`s syndrome from Korea in the medical literature. A 56-year-old woman was referred to our hospital with cough productive of sputum and chest tightness. The Computed tomography scans of the chest revealed multiple and well-defined peribronchiolar nodular opacities. A video assisted thoracoscopic surgery (VATS) biopsy was performed and the nodular opacity in the lung parenchyma was pathologically confirmed as NLH. Through meticulous review of patient`s record, we found that she had been suffering from dry eye and dry mouth. The symptoms suggested Sjogren`s syndrome, which was confirmed by specific laboratory tests including the Schirmer test, anti-nuclear antibody and anti-Ro/La antibody. The patient is followed regularly and has no further progression of symptoms.