The case report describes two cases of siblings who presented with short root anomaly with oligodontia, dens invaginatus, taurodontism, pulp stones, multituberculism of molars and generalized microdontia. No familial occurrence was seen and the dental anomalies were not associated with systemic manifestations. The sporadic occurrence of a combination of dental anomalies in a single patient can present a diagnostic challenge due to the possibility of their occurrence with various syndromes and systemic conditions. The article also reviews previously reported cases of short root anomaly with other dental anomalies which showed diverse variations in the pattern of clinical presentation. Treatment plan in both the siblings was aimed at preserving existing teeth and early management of oligodontia.