The cribriform-morular variant of papillary thyroid carcinoma (CMV-PTC) is now designated as morular cribriform thyroid carcinoma (CMTC) according to the 5thedition of the World Health Organization (WHO) Classification of Thyroid Tumors. CMTC can appear within a familial adenomatous polyposis (FAP) or be sporadic. We report the first case of a young female patient in China who was diagnosed with FAP and CMTC with a mutation in exon 16 of the APC gene underlying the disease. The main purpose of this case report is to provide a special pathological type of thyroid tumors, which is expected to be helpful for clinical work in the future.