A 07-months-old boy, from a non-consanguineous marriage, born by cesarean section, with a birth weight of 3300g, unique of his family.

      He has a familial history: Her mother has a malformation of the fingers with a well tolerated trabecular ventricular communication, acute rheumatoid arthritis treated with extencillin until the age of 18.

      The history of the disease dates back to the age of 3 months with the appearance of cyanosis during feedings complicated by dyspnea, a heart murmur was found on physical examination, a chest X-ray was made objectifying a cardiomegaly supplemented by a transthoracic ultrasound which showed a complete atrioventricular communication,

      Due to the association of an anomaly of the upper extremities made of a triphalangeal thumb, phocomelia, radial aplasia, shortening of the left upper limb, with congenital heart disease, the diagnosis of holt oram syndrome was retained.

Figure 1: Triphalangea with radial aplasia.

Figure 2: Chest X-ray objectifying a cardiomegaly.