Fistulas between systemic and pulmonary arteries are associated with various underlying etiologies and cause pulmonary hypertension (PH). Diagnosis of this condition requires several imaging studies and the exclusion of other possible causes of PH. We herein report a case of a patient with interstitial pneumonia and scleroderma. The imaging revealed multiple fistulas involving the inferior phrenic and left lower pulmonary arteries. The fistulas were closed using coils, but the PH remained presumably due to other undiagnosed fistulas. The improvement of symptoms following use of a supplementary pulmonary vasodilator provides the hope that the chosen treatment could be a viable alternative approach for other similar cases.