ABSTRACT Pulmonary artery hypertension (PAH) is a rare initial presentation of connective tissue disorder (CTD) like systemic lupus erythematosus (SLE). SLE associated with PAH has worse prognosis than isolated SLE. However, there has been improvement in mortality of the patients in the recent years due to newer treatment modalities. Still early recognition is the key step. Here we present a case of young female with pulmonary hypertension associated with right heart failure and on further investigation was found to have CTD associated PAH. This case represents the importance of early recognition and treatment of SLE-associated PAH which will improve the survival rate in the patients. Keywords: Pulmonary artery hypertension, systemic lupus erythematosus, connective tissue disorder