A 65 year-old female with a history of xerostomia and xerophthalmia was presented with dyspnea on exertion (New York Heart Association class III). Echocardiography and cardiac catheterization demonstrated severe pulmonary hypertension (PH). Laboratory examinations showed positive anti-nuclear and anti-Ro/SS-A antibodies. Schirmer's test was positive and salivary gland scintigraphy revealed severely decreased tracer uptakes in both parotid and submandibular glands. By excluding other possible causes of PH during further examinations, she was diagnosed with severe PH associated with primary Sjogren's syndrome. Her dyspnea symptom was much improved with endothelin receptor antagonist and azathioprine.