A 40-year-old female presented with cough, exertional dyspnea, abdominal pain with distention, fatigue, dry eyes and dry mouth. Past history revealed asthma. Physical examination was normal except for tachypnea. We found leukocytosis, azygos fissure on chest X-ray along with normal pulmonary function tests and arterial blood gases. Thorax computed tomography (CT) revealed bronchiectasis and ground glass opacities in both lungs. Abdominal CT demonstrated thrombosed proximal splenic artery aneurysm. Further diagnostic procedures were done and according to the positive Schirmer test and compatible histopathologic findings of the salivary gland, diagnosis of primary Sjögren’s syndrome was established. Splenic artery aneurysm is rare occurring in less than 1% of the population that usually appears as an incidental finding. This is the first case in literature that introduces Sjögren’s syndrome as a risk factor for splenic artery aneurysm. The silent presentation of the splenic artery aneurysm should previse the clinicians that such an occurrence may cause a significant diagnostic dilemma.