Adrenal gland disorders can be asymptomatic and detected incidentally via imaging techniques such as ultrasound, computed tomography (CT), positron emission tomography, and magnetic resonance imaging. Fusion anomaly is a condition that can be attributed to errors in the developmental process and may be detected via these imaging modalities. We present a case of butterfly adrenal gland in a 61-year-old man with CT and magnetic resonance images. In our patient, this anomaly is also accompanied by unilateral renal agenesis and a diaphragmatic defect. Positron emission tomography-CT, contrast-enhanced CT, and magnetic resonance images are presented. To the best of our knowledge, this is the second case in which coexistence of unilateral renal agenesis and butterfly adrenal gland anomaly in an adult patient has been documented.