Introduction. Coloboma is a Greek word, which describes the defect of all layers of the organ, and it can be congenital or as the result of an injury, operation, or some disease. Congenital upper eyelid coloboma is a rare anomaly, with the unknown incidence. The size of the defect is different, but it always involves all layers of the eyelid. This malformation is more frequent at the upper eyelid, and unilaterally, at the junction of the medial two thirds. Sometimes, it can also involve the eye, and may be a component of many syndromes (Goldenhar, Fraser, Manitoba, CHARGE, Cat eye). Case report. We are describing the case of the upper eyelid coloboma with the rare eyebrow anomaly at the three-month old girl, and the result of reconstruction. The baby was treated conservatively with lubricants and overnight patching. Pentagonal excision of the defect was performed in general anesthesia. Three layers of the eyelid were prepared: the skin, muscle and tarsoconjunctival layer. Because of orbicularis muscle malposition, reinsertion and reposition of the muscle fibres were performed. Then, lateral canthotomy was made and the suture of three layers of the eyelid. Catgut suture 7-0 was used for the conjunctiva and muscle. Nylon 6-0 was used for skin suture. Z-plasty was done on the upper part of the pentagonal excision in order to reduce skin tension at the suture line. The operation lasted about 60 minutes and the hospitalization three days. The occlusive dressing was applied for two days. The stitches were removed after seven days. The postoperative swelling of the upper and lower eyelid disappeared in five days. There were no complications in the postoperative period. Conclusion. The main principle of the treatment of eyelid coloboma is surgical reconstruction of all layers of the eyelid, in optimal period, using different surgical methods, which depends on the size of the defect. An early diagnosis is of the greatest importance, as well as the treatment of associated anomalies. Complications of the upper eyelid coloboma depend on the size of the defect, presence of the eye anomalies and the method of reconstruction.