This paper is a case report of a 10-year-old girl with focal dermal hypoplasia (FDH) who presented for dental care. She displayed many of the well-documented oral features associated with this syndrome. Additionally, she had taurodontism of a permanent mandibular molar. Although taurodontism affected only one tooth in this patient, the presentation of taurodontism with FDH has not been reported...

Differential diagnosis of NLCS includes nevus sebaceous, skin tag or fibroepithelial polyp, neurofibroma, lymphangioma, lipofibroma, focal dermal hypoplasia (Goltz syndrome), lipomatosis, and Michelin tire baby syndrome.[2,4] Histopathological examination helps in the differentiation. There is absence of fat cells in the dermis in skin tag. In lipofibroma skin, appendages are absent in the derm...

Focal dermal hypoplasia (FDH) is a rare disorder of the mesodermal and ectodermal tissues. Here we present an eight-year-old female known to have FDH who presents with poor weight gain and dysphagia. She was diagnosed with multiple esophageal papillomas and eosinophilic esophagitis. She was successfully treated with argon plasma coagulation and ingested fluticasone propionate, which has not bee...