Background: Ackerman’s syndrome is the rarest of the rare syndromes with a documented prevalence rate of less than one in a million cases1. This syndrome has been known to present with Pyramidal molars, abnormal upper lip, wide philtrum, clinodactyly or syndactyly and sometimes also associated with juvenile glaucoma. Pyramidal molars are essentially single rooted molars, a rare morphological variation from the usual multi rooted molars and have very rarely been reported in the deciduous dentition. It is said to occur due to the failure of invagination of the Hertwig’s epithelial root sheath.
Case report: Patient had reported to the department and a detailed examination and radiographic co-relation revealed the possibility of Ackerman’s syndrome.
Conclusion: This is a report of a case of Ackerman’s syndrome with pyramidal first deciduous molar in an eight year old female patient diagnosed by chance.