We present a case of a 34-year-old male with a unilateral papillary cystadenoma of the right epididymis (PCE). His past medical history is remarkable for Von Hippel–Lindau disease (VHLD), bilateral renal clear cell carcinoma, and hemangioblastomas. The patient noticed an abnormality with mild tenderness in the right scrotum. Ultrasound (US) of the right scrotum demonstrated a solid and cystic epididymal lesion. In this case report, we present the sonographic features of the PCE in the setting of a patient with VHLD.