Pulmonary hypertension may be associated with many clinical conditions, including connective tissue diseases. We present a case of a patient with sclerodermia and chronic myeloid leukemia, who developed severe symptoms of pulmonary hypertension. Atypical clinical course of pulmonary hypertension, including complete remission of clinical symptoms and hemodynamic improvement provoked critical approach to the etiology of pulmonary hypertension. Taking into account the temporal coincidence with the use of dasatinib (a tyrosine kinase inhibitor) and a few case reports in the literature, it appears that reversible pulmonary hypertension in our patient was associated with the use of dasatinib. Sclerodermia in a previous medical history, an acknowledged risk factor for pulmonary hypertension, initially delayed the correct diagnosis. Reclassification changes the clinical prognosis of pulmonary hypertension in this specific case and allowed to terminate of specific treatment of pulmonary hypertension with good results.