Managing pericardial effusion in autoimmune diseases like systemic lupus erythematosus becomes particularly challenging when accompanied by pulmonary hypertension (PH). The risk of acute right ventricular overload and hemodynamic collapse makes pericardial drainage a high-stakes decision. This case highlights the necessity of a multidisciplinary, individualized approach in high-risk patients. It also underscores the effectiveness of immunosuppressive therapy (e.g., mycophenolate mofetil) and pulmonary vasodilators (e.g., Ambrisentan, Tadalafil) in achieving gradual resolution. Close collaboration with PH specialists is essential for safely navigating these complex cases.