Congenital Bochdalek hernia (BH) in an adult is rare and has an unusual presentation. They are confined to the pediatric age group with an incidence of 1:3,000 live births. It rarely persists asymptomatic until adulthood. Surgical repair by thoracic, abdominal, or thoraco-abdominal approach is the treatment of choice with diaphragmatic reconstruction in associated diaphragmatic agenesis. With only 10 cases of BH with partial diaphragmatic agenesis reported to date, we discuss the rarity, unusual presentation, and management of BH in a young adult with sickle cell disease that has not been reported in the literature.