These include the second reported case of intracranial germ cell tumor in an APC carrier19, absence of profuse polyposis and early onset CRC in 3 of the 7 codon 1309 mutation carriers as is classically described20, attenuated phenotype with only 50 polyps at age 33 years in a codon 593 mutation carrier, desmoid tumor with codon 1228 mutation, papillary thyroid cancer with codon 1346 mutation and most interestingly CHRPE with codon 1483 mutation7, 8. The gene discussed is APC; the disease is colorectal carcinoma.