Abstract
Only 20-30% of the 300 cases reported have exhibited the classical features of obesity, retinitis pigmentosa, polydactylism, mental retardation, hypogonadism and familial incidence. The present case is the first description of the syndrome in an Arab boy with all the foregoing features. The pedigree is presented to show the role of consanguinity and the incidence of the syndrome in 2 siblings of the patient''s grandparents.

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