Aplasia Cutis Congenita (ACC) is a rare disorder characterized by the congenital absence of a portion of skin. The disease may involve any site of the body, preferentially the vertex of the scalp. More than one single cause is considered as responsible for the aetiology of the disease, including vascular anomalies. If the defects are large or deep, especially on the vertex, surgical intervention is necessary. We report on a 5 year-old ACC case that healed spontaneously after birth and, was admitted to our hospital for a 12 × 13cm cicatricial alopecic area and skull defect on the vertex. For treatment, expanded scalp flaps were planned and applied after a non-problematic expansion for a long period. The expanded scalp flap was transposed to the defect and although there was no tension, venous insufficiency developed and the flap was lost during the post-operative period. Immediately after this, another transposition flap was used to close the skin defect. Again, partial flap failure with venous insufficiency developed. A split skin graft was applied after granulation tissue developed on the site ofdebrided necrotic areas. The final result was partially satisfactory with alopecic areas due to skin graft instead of scar that could have resulted from the spontaneously healed skin defect and preserved bone grafts. We suggest that the flaps used for reconstruction of ACC defect in the scalp should be either advancement or bipedicled flaps, because of better reliability.