Pulmonary arterial hypertension is a rare disease but it has gained increasing attention due to its high mortality risk. Here, we presented a case of connective tissue disease related pulmonary arterial hypertension. In this case, it highlights the complex etiology of pulmonary arterial hypertension in relation to connective tissue disease. This patient had both components of group 1 and group 3 pulmonary arterial hypertension. It is important to risk stratify the patient, to consider escalation of therapy including combination therapy when treatment goal of low risk not achieved. Multidisciplinary approach from cardiology, rheumatology and pulmonology as well as shared decision between patient and attending physician do play a crucial role here.