A 43-year-old male presented with multiple (>10) painful, papulo-nodular lesions, associated with paresthesia, over the face, neck and trunk, appeared 15 years prior and increased with time. The patient's brother has cutaneous leiomyomas and testicle cancer, his mother underwent hysterectomy for symptomatic leiomyomas while his maternal grandfather died from aggressive renal cell carcinoma at age 57 years. The patient reported that he had removed a kidney cancer 5 years prior. Dermoscopy, histologic evaluation and genetical analysis were done and Hereditary leiomyomatosis and renal cell carcinoma was diagnosed.