Dentinogenesis imperfecta represents a group of hereditary conditions that are characterized by abnormal dentin formation. These conditions are genetically and clinically heterogenous and can affect only the teeth or can be associated with the condition osteogenesis imperfecta. It is an uncommon defect in the collagen formation that is transmitted as an autosomal dominant trait. This case report discusses the dental manifestations of DI in a 4-year-old. Rigorous home care instructions, including reinforcement of the oral hygiene practice were discussed with the parents.