Purpose: To evaluate craniofacial and occlusal features of children with sickle cell disease and compare them with the normal healthy children. Methods: A total of 50 children diagnosed with sickle cell disease (Group I) and 50 normal healthy children (Group II) between age 10 and 18 years were included in the study. Dental casts were obtained, occlusal traits were recorded and DAI and DHC-IOTN were calculated. Cephalometric parameters were measured. Obtained data were compared between the two groups. Results: In children with sickle cell disease, 24% had definite malocclusion according to DAI and only 4% of them had normal occlusion as per the DHC-IOTN index. Also, children with sickle cell had significant retruded mandible and vertical growth pattern when compared with normal children. Conclusions: Children with sickle cell disease showed delayed eruption of teeth, a tendency towards Class II molar relationship, with increased crowding in the lower anterior region, increased overjet and open bite when compared to the normal children. Children with sickle cell disease had severe and handicapping malocclusion as per DAI and DHC-IOTN indices and a tendency towards skeletal Class II with a vertical growth pattern. © 2019, European Academy of Paediatric Dentistry.