Lenz-Majewski Hyperostotic Dwarfism (LMHD) is an extremely rare congenital, sclerosing bone dysplasia that causes cranio-tubular hyperostosis, ectodermal dysplasia (cutis laxa and enamel hypoplasia), osseous dysgenesis of hands and feet with diaphyseal cortical thickening of tubular bones and intellectual disability.
Keywords: Lenz-Majewski Hyperostotic Dwarfism, Lipoma, Obstructive Hydrocephalus.
Differential Diagnosis: 1) Ehlers--Danlos syndrome, 2) Gerodermia osteodysplastica 3) Lenz Majewski Syndrome, 4)
Hyperostotic dwarfism, 5) SCARF Syndrome (Skeletal abnormalities, Cutis Laxa, Craniostenosis, ambiguous genitalia, retardation, facial abnormalities, Costello syndrome) and 6, MACS syndrome (Macrocephaly, Alopecia, cutis laxa and scoliosis).