Green pigmentation in teeth is an uncommon condition associated with bilirubin deposits in hard dental tissues. Its occurrence can cause anxiety to both the child and parents and is not diagnosed easily by clinicians. The aim of this study is to analyze the current knowledge about the etiology, the intraoral alterations, and the macroscopic and microscopic features of green teeth pigmentation related to a high bilirubin levels. A primary tooth was extracted and manually sliced into 600 microns thin sections. The slenderized slices were examined with a light microscope AxioImager M1 to evaluate the microscopic teeth structure. The clinical characteristics of teeth may help in the diagnosis of current or past systemic diseases. Pediatricians should be able to quickly note the signs in order to perform the proper diagnosis. This study may help clinicians gain more knowledge about the current status of this uncommon pathology.