Background: Fanconi's Anemia (FA) is a rare, inherited autosomal recessive genetic disease that causes aplastic anemia.The incidence of the disease is unusually higher in South Africa with calculated heterozygote prevalence being approximately 1 in 77. The male-to-female ratio in the literature cases is 2:1. Objectives: This report describes the oral clinical and radiological findings in a 16 years old male of mixed ancestry with FA. Methods: Medical and dental history was noted, the oral mucosa was examined and the teeth were screened for fillings and caries. Periodontal examination consisted of: measurement of probing depths, recession, mobility of teeth, furcation involvement, plaque index (Loë & Sillness) and gingival index (Sillness &Loë). A panoramic radiograph was taken. Results: There was a generalized black hyperpigmentation on the buccal mucosa, tongue and palate. The patient had his 46 tooth extracted. He had multiple fillings at 16 15 14 24 26 36 and 37. The teeth 17 15 14 36 35 34 were carious. The patient had moderate to severe generalized gingival recession and most of the periodontal pockets were deeper than 5mm. The patient had generalized grade two mobility and loss of alveolar bone in the furcation areas of most posterior teeth. The mean gingival index was 2.1and the mean plaque index was 2.3. Panoramic radiograph showed severe generalized horizontal bone loss. The patient was diagnosed with severe generalized periodontitis associated with systemic disease. Conclusions: Patients with Fanconi's anemia are susceptible to severe dental and periodontal diseases. Key Words: Oral mucosa; Fanconi's anemia; periodontitis; hyperpigmentation; caries; autosomal recessive.