Agenesis of one or more teeth is a common finding in both primary and permanent dentition. The most commonly missing tooth in permanent dentition is the third molars, followed by the maxillary lateral incisors or mandibular premolars. Congenitally missing maxillary central incisors are not frequently found. The presence of only one maxillary central incisor is a rare clinical finding called a solitary median maxillary central incisor. This case report describes a 7-year-old female patient who presented with only one central incisor at the midline. Early diagnosis of the condition is essential to rule out other associated problems.