Knowledge on variations in roots and canal morphology of teeth is very important for successful endodontic treatment. Most of the times, permanent mandibular first molar (PMFM) usually have two roots one mesial and one distal with three root canals. Although variations in root and canals of this tooth have been extensively discussed in the endodontic literature, existence of a single root with a single canal is not well-documented. The aim of this report is to present an unusual anatomy of the PMFM having a single root and single canal along with other peculiar multiple anomalies such as tooth agenesis, single-rooted maxillary first molars and three rooted primary mandibular second molar in the same patient.