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A case of mutiple nevoid basal cell epithelium, cysts of the jaw, and bifid rib syndrome has been reported because of the large number of positive findings in a single patient. Symptoms were a simple manner with slow learning ability, frontal bossing, wide nasal root, hypertelorism, large superciliary ridge, deep-set eyes, bifid uvula, dentigerous keratocyst of the mandible, pectus excavatum, bilateral shortened metacarpals, multiple basal cell nevi, bilateral palmar pitting, hemivertebrae C2-3 and C7-T1, several bifid ribs, calcification of the falx cerebri, bridging of the sella turcica, and an open frontal suture. The need for establishing diagnosis has been emphasized.
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