Abstract
Carney complex (CNC) is a rare tumor syndrome caused by mutations in the PRKAR1A gene, characterized by endocrine tumors, cardiac and cutaneous myxomas, pigmented lesions, and testicular tumors. One to two thirds of affected males develop large cell calcifying Sertoli cell tumors (LCCSCTs), with rare involvement of scrotal soft tissues. We present a case of CNC in an adolescent male with bilateral LCCSCTs and a very large scrotal myxoma, resulting in azoospermia and pain requiring surgical management. This report highlights the urologic manifestations of CNC and outlines appropriate evaluation, treatment, and surveillance strategies relevant to pediatric and adolescent urologic practice.