We will focus on infarct and thromboembolic disease, vasculitis, vascular malformations, and pulmonary hypertension. The classification for vasculitis has changed, which causes problems in pulmonary pathology, as often the diagnosis is made on tissue analysis. For example, granulomas are not seen in about 70% of Wegener’s granulomatosis, which would result in shifting these cases into microscopic polyangiitis, despite other data would not fit with this entity. Therefore, we keep both classifications, which might create less confusion for our clinical colleagues. Pulmonary hypertensive diseases will not often be subjected to pathological analysis; however, the pathologist might be confronted in autopsy cases or in rare diseases with hypertension. Therefore, we will describe the morphological changes to be seen in histological specimen to enable the pathologist to make the diagnosis of pulmonary hypertension and some variants. In some cases, such as in systemic diseases or in autopsy cases with heart diseases the pathologist can arrive at a diagnosis, which includes also the etiology of hypertension, but in biopsies the final diagnosis has to be established by a multidisciplinary team led by the clinician.