In the first MRI examination performed upon our patient, a poorly demarcated area (2.0 × 3.5 × 5.0 mm) was described in the anterior part of the glandular lobe; in the examination performed 6 months later, this area maintained poor visibility, while laboratory results at the time clearly indicated an ACTH-dependent form of CS. Here, POMC is linked to Cowden syndrome 1.