Vol. 6, Issue 1, Part C (2022)

Abstract: A rare syndrome characterized by the association of typically unilateral, closely arranged, linear, slightly elevated, multiple, nevus comedonicus lesions located usually on the face, neck, trunk and both limbs with extracutaneous presentation within the variety of syndactyly, polydactyly, valgus deformity in lower limb and scoliosis. Our case presented with additional findings of bilateral congenital inguinal hernia with atrophied right testis and retractile left testis, which has not been mentioned before.