Happle-Tinschert syndrome is a disorder causing unilateral segmentally arranged basaloid follicular hamartomas of the skin associated with ipsilateral osseous, dental and cerebral abnormalities including tumors. Although a case with hemimegalencephaly was previously described, this is the first report of Happle-Tinschert syndrome with discrepant short left leg, ipsilateral skin lesions, hemimegalencephaly and frontal polymicrogyria.
Basal Cell Nevus Syndrome/diagnosis ; Child ; Dental Enamel/abnormalities ; *Frontal Lobe/pathology ; Hamartoma/*diagnosis/pathology ; Humans ; Leg Length Inequality/*diagnosis ; Magnetic Resonance Imaging ; Male ; Malformations of Cortical Development/*diagnosis ; Skin Diseases/*diagnosis/pathology ; Syndrome