McCune-Albright Syndrome (MAS) is characterized
by a triad of physical signs-café-au-lait pigmented
skin lesions, polystotic fibrous dysplasia (FD) &
endocrine dysfunction, which particularly manifests
as peripheral precocious puberty in girls.1,2 It is a
rare disease with an estimated prevalence between
1/100,000 and 1/1,000,000.Thecafé-au-lait skin
pigmentation consists of large hypermelanotic
maculae of irregular and serpiginous (coast of
Maine) borders which occur mainly on the front,
posterior area of neck, buttocks, thorax, back,
shoulder and pelvis.3 FD is a benign condition in
which the medullary portion of the bone is replaced
by poorly organized fibrous tissue with trabeculae
of immature bone. It may affect either a single bone
(monostotic) or numerous bones (polyostotic). It is
caused by embryonic somatic mutation, leading to
the substitution of His or Cys for Arg at aminoacid