Arginine vasopressin deficiency and sellar/cutaneous xanthogranuloma: A rare association

Sellar xanthogranuloma is a rare cause of idiopathic arginine vasopressin deficiency (AVP-D). We are reporting a case of AVP-D and cutaneous and sellar xanthogranuloma. The patient was diagnosed with AVP-D 11 years ago, but sellar magnetic resonance imaging was not done at presentation. Patient presented with cutaneous xanthogranuloma over face 2 months back. During endocrine work-up, central hypothyroidism and possible growth hormone deficiency were found. Sellar imaging revealed intrasellar mass with imaging characteristics of xanthogranuloma without mass effects. Xanthogranuloma should be in the differential diagnosis of idiopathic AVP-D and sellar masses, and a detailed skin examination should also be done.