Idiopathic pulmonary arterial hypertension (IPAH) is a rare but devastating disease that most often affects women in their 30s, and the diagnosis is usually based on exclusion. Minimal change disease (MCD) is the primary cause of nephrotic syndrome in children younger than 16 years of age.We report an unusual case of a 47-year-old male with MCD who was found to have IPAH. The patient’s age, gender, initial clinical manifestations, and the unusual coexistence of both MCD and IPAH complicated the process of arriving at an accurate diagnosis.