Background Progressive familial intrahepatic cholestasis is a heterogeneous group of disorders, leading to intrahepatic cholestasis, with the possibility of chronic liver failure and biliary cirrhosis. Oligodontia is either the manifestation of a specific syndrome or is non-syndromic. To the best of our knowledge, this is the first case report of type 3 progressive familial intrahepatic cholestasis and concurrent oligodontia, craniosynostosis, dens in dente, taurodontism, and delayed permanent dentition in the medical and dental literature. Case presentation We present the dental and medical histories and comprehensive dental management of a girl with type 3 progressive familial intrahepatic cholestasis and several dental anomalies, who was referred to a dental clinic due to severe dental caries and pain. Conclusion Our findings suggest that PFIC with manifestations as oligodontia, craniosynostosis, dens in dente, taurodontism, and delayed permanent dentition, might indicate an unknown syndrome; otherwise, the craniofacial anomalies are the manifestations of an independent disease coinciding with PFIC. Moreover, our case is a good example of the importance of timely medical and dental care in confining further health-related complications. The patient was able to ingest without any pain or discomfort after receiving proper dental management.

Objective Given the importance of preserving caries‐affected dentin (CAD) in conservative dentistry, the shear bond strength (SBS) of different resin cements to CAD has been investigated. Here, we aimed to compare the SBS and remineralizing effect of a calcium silicate (TheraCem) and conventional self‐adhesive cement (Panavia SA) on the SBS of CAD. Materials and Methods Forty‐eight extracted third molars (24 sound and 24 CAD) were used. In each group, 12 teeth were prepared for bonding to TheraCem or Panavia SA. After removal of the enamel and caries, resin composite cylinders were luted on the prepared dentin. After 28 days of storage in the artificial saliva, SBS was measured and the failure mode analysis was investigated. The images of fractured sections were analyzed using scanning electron microscopy and energy‐dispersive X‐ray to evaluate the Ca/P weight ratio. Results SBS of CAD and sound dentin was not different when cemented with TheraCem (9.56 ± 4.51 vs. 9.17 ± 2.76, p = .806), but the CAD showed significantly lower SBS to Panavia SA (9.4 ± 2.36 vs. 7.39 ± 2.18, p = .015). The Ca/P ratio in CAD was significantly higher when bonded to both TheraCem and Panavia‐SA than that of the controls (p = .001); however, this ratio was not different for those bonded to TheraCem compared to Panavia SA. Conclusions Based on our results, TheraCem as a calcium silicate cement shows better SBS to attach the restoration to CAD as compared to Panavia SA. Obliteration and mineralization of the dentinal tubules in TheraCem were also higher than in Panavia SA. However, their ability to improve the amount of the Ca/P ratio in CAD was similar.