Dyskeratosis congenita is a severe, multisystem bone marrow failure syndrome, with associated cutaneous and noncutaneous abnormalities. The triad of abnormal skin pigmentation, nail dystrophy and mucosal leucoplakia characterize dyskeratosis congenita. Other findings include learning difficulties or mild-to-moderate mental retardation, short stature, underdeveloped or undescended testes, and abnormal bone trabeculation or osteoporosis. Dyskeratosis congenita is a rare syndrome, with approximately less than 200 individuals reported in the literature, with 90% of patients being male. Dyskeratosis congenital is a serious and usually fatal disease. Death may occur due to carcinoma, hemorrhage, and sepsis.