A middle aged lady presented with gradual increase in shortness of breath and reduced vision bilaterally. The patient had no history of Diabetes mellitus, hypertension or any other co-morbid illness. Examination revealed signs of right sided heart failure, pulmonary hypertension and neovascularization of retina. Echocardiography confirmed pulmonary hypertension and fluorescence angiography confirmed neovascularization of retina. Treatment with Sildenafil resulted in symptomatic improvement.