Abstract
Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a rare genetic disorder, and individuals tend to develop multiple cutaneous leiomyomas, uterine leiomyomas, and renal cell cancer (RCC).
In our study, we report the first case in Saudi Arabia, to our knowledge - a 28-year-old male with a history of right leg leiomyosarcoma post excision two years back who was referred to us with incidental finding of right kidney mass measuring 1.8x2x2.2 cm who underwent right laparoscopic radical nephrectomy, and histopathology reported it as HLRCC and RCC.