Ectopic Adrenocorticotropic Hormone–Producing Renal Cell Carcinoma Causing Psychosis in a Patient With Birt-Hogg-Dubé Syndrome

This is, to our knowledge, the first-ever recorded case of Birt-Hogg-Dubé syndrome associated with an adrenocorticotropic hormone–secreting renal cell carcinoma. A 35-year-old woman who presented with new-onset psychosis, hypertension, and diabetes was found to have severe hypokalemia and elevated cortisol and adrenocorticotropic hormone levels. Imaging revealed a renal mass, pathology confirmed chromophobe renal cell carcinoma, and genetic testing confirmed Birt-Hogg-Dubé syndrome. The patient underwent right partial nephrectomy, with resolution of her symptoms reported.