Multiple cutaneous and uterine leiomyomatosis (MCUL), previously known as Reed's syndrome, is an autosomal dominant disease with incomplete penetrance characterized by the onset of uterine leiomyomas or fibroids and skin leiomyomas in both sexes [
1,
2]. The coexistence of cutaneous and uterine leiomyomas may cluster papillary type2 renal-cell carcinoma or renal collecting duct cancer, codifying a recent variant appointed as hereditary leiomyomatosis and renal cell cancer (HLRCC) [
3]. Even though different gene mutations, missense, nonsense or whole gene deletions have been described in MCUL syndrome, a definite association between site or type of mutation and the risk of papillary renal-cell carcinoma has not been found yet [
4]. Cutaneous leiomyomas - accounting for 75% of all extra-uterine leiomyomas - are uncommon benign smooth muscle tumours deriving either from the erector pili muscle of the pilosebaceous unit (piloleiomyomas), the cutaneous vascular smooth muscle fibers (angioleiomyomas) or from the dartos muscle (genital leiomyomas) [
5]. Piloleiomyomas are the most common form and show firm, skin-colored or pink-brown soft dermal papules or nodules, ranging up to 2.0 cm in diameter, distributed in single, clustered, segmental or disseminated patterns. Linear or segmental leiomyomas may present a dermatomal-like distribution [
6]. Common locations include trunk, extensor surface of the extremities and face, more often in adulthood then in childhood. Neoplasms can be asymptomatic or quite debilitating and actually painful mainly in response to pressure or cold temperature [
7]. Clinical differential diagnoses include several skin lesions, such as dermatofibroma, eccrine spiradenoma, neurofibroma, angiolipoma, neurilemmoma, glomus tumour, keloid, hamartomas and blue rubber bleb nevus syndrome when painful, or dermal nevus, trichoepithelioma, lipoma, cylindroma or poroma among the asymptomatic tumours [
8]. The skin involvement of a case of MCUL syndrome is reported to support the diagnostic role of the cutaneous counterpart in identifying this rare syndrome. We present and discuss a case of multiple cutaneous lesions and the usefulness of dermoscopy for a better understanding of skin leiomyomas in the wide spectrum of non-melanocitic lesions.