BACKGROUND: A 57-year-old African American man presented to a tertiary care center with a 6-month history of fatigue and worsening abdominal pain. He had lost 9.1 kg in weight in the 3 months leading up to presentation, and described subjective fevers and night sweats for 1 month with a nonproductive cough and blurred vision for several weeks before presentation. He had chronic renal insufficiency, sleep apnea, hypertension, and peripheral vascular disease. INVESTIGATIONS: Physical examination, complete blood count, peripheral blood smear, leukocyte alkaline phosphatase score, bone marrow biopsy, CT of the chest, abdomen, and pelvis, MRI of the abdomen and pelvis, measurement of plasma and urine metanephrines, 24 h urine testing for cortisol and 17-ketosteroids, measurement of serum granulocyte colony-stimulating factor (G-CSF) level, histopathologic examination and immunohistochemical staining of resected tumor. DIAGNOSIS: G-CSF-secreting adrenal carcinoma with rhabdoid-like differentiation. MANAGEMENT: En bloc surgical resection of kidney, suprarenal mass and spleen was performed, followed by initiation of mitotane chemotherapy 3 months later.