Talon cusps, macrodontia, and aberrant tooth morphology in Berardinelli-Seip syndrome

Berardinelli-Seip syndrome is a congenital form of generalized lipodystrophy, transmitted as an autosomal recessive trait. It is well documented in medicine and skin. It is a rare disorder caused by mutations of AGPAT2 gene or BSCL2 gene. We present an interesting case of Seip-Berardinelli syndrome in female siblings aged 12 years and 14 years who presented with endocrine disturbances along with dental manifestations including talon cusps, macrodontia, aberrant tooth morphology, and severe generalized crowding. The presence of such dental abnormalities may represent an interesting association with endocrine disturbances. Such cases require thorough investigations and appropriate treatment.