The incidence of pulmonary arterial hypertension (PAH) is increasing day by day. Newer advances in the management of PAH are being developed. The efficacy and safety of these agents is monitored by applying these in clinical practice. Here is a case of a 34yr old female diagnosed with severe PAH, RV dysfunction, heart failure with multiorgan dysfunction, diabetes mellitus and congestive heart failure. She was on sildenafil and ambrisentan+tadalafil combination for PAH. Initially ambrisentan was withheld due to complaints of fluid retention. Later macitentan which is known to cause comparatively less edema was started as a substitute for ambrisentan along with sildenafil. After 10 days patient developed generalised edema and weight gain of about 10kg. The skin developed orange peel- like (peau'd orange) appearance. Macitentan was withheld on the following day and the problem was resolved. Her clinical condition improved, she was ambulated and discharged on O2 support. Need for a vigilant monitoring when using newer drugs is very essential for improving patient safety.