Aim: The aim of this article is to report a case of green discoloration of primary teeth associated with neonatal cholestatic jaundice and sepsis in a 2-year-old girl. Background: Systemic changes can lead to dental alterations of the teeth. One of the disorders is elevated serum bilirubin levels that can cause pigmentation of teeth. Green pigmentation of teeth is uncommon, but when it occurs, is a cause of concern for parents and the child, as he may also loose self-confidence. Case description: The parents reported to the clinic with the child whose chief complaint was green pigmented teeth. Her medical records revealed she was preterm and was diagnosed with neonatal cholestatic jaundice which lead to hyperbilirubinemia and sepsis. On review at 36 months of age, all her deciduous teeth were completely erupted and a striking demarcation was visible between the green incisal edges and the normally colored cervical one-thirds of all primary teeth. Treatment alternatives were suggested and the child was kept on regular follow-ups. Conclusion: So, one should be aware of such condition and seek medical practitioner or reports to confirm the diagnosis. If such a condition exists, esthetic treatment can be achieved with the newer techniques in cosmetic dentistry. Clinical significance: Green pigmentation has very limited causes. The time of illness coincides with the stage of tooth formation. Proper medical history is important to evaluate the extent of pigmentation and probable involvement of the permanent teeth.