Endocrine manifestations of the antiphospholipid syndrome (APS) are very rare. Adrenal insufficiency, surprisingly usually due to venous infarction, is the most common. It can even be the presenting manifestation of APS, occur in patients with transient antiphospholipid antibodies or even in a neonate through transplacental transfer of maternal antiphospholipid antibodies. The presentation, differential diagnosis, and treatment of adrenal involvement due to APS will be reviewed. Other even rarer endocrine manifestations will also be briefly discussed.