Herein, we describe the interesting case of an SS-PAH patient who had PVOD and carried a novel biallelic EIF2AK4 mutation at c.1021 C > T (p. Gln341*); we also noticed that PAH-targeted therapies were well tolerated by our patient and did not cause pulmonary edema after following the patient for a three-year follow-up period. The gene discussed is EIF2AK4; the disease is pulmonary venoocclusive disease.