Congenital skeletal limb abnormalities refer to a variety of bone structure problems in the arms or legs (limbs) due to congenital or inherited causes. From the Genetics Clinic of Cairo University Children's Hospital 44 selected cases were studied. Malformations were present in 7 cases (16%), deformation in the form of talipes equino varus in 6 cases (13%), disruption with the terminal transverse limb reduction defect in 4 cases (9%), and sequence in 1 case (2%). 13 cases (30%) had lower limbs affection, 8 cases (18%) had upper limbs affection, while 23 cases (52%) has both upper and lower limbs affected. No exposure of drugs or chemicals or infections in our cases.