AIM: Diagnosis and treatment of tumors of the eye is extremely difficul; surgical treatment in advanced stages, when the tumor grows in the orbit, leads to extensive radical surgery of the face. The extent and nature of surgical procedures depends on the nature of the tumor process, in advanced stages is indicated mutilating surgery--exenteration of the orbit. Exenteration of the orbit due to the extrascleral extension of malignant melanoma of the uvea is very rare, unfortunately, even today in certain cases it is necessary to make such a mutilating surgery. MATERIALS AND METHODS: Case report--65 year old female patient, sent to our Departement in 2008 with the finding of the pigment deposits on the posterior pole of the left eye. Ultrasound study found elevations of up to 3 mm, she was asked to come for further control in three months interval. She did not coma, furthermore she sporadically attended another eye clinic. In 2011 she was treated for secondary glaucoma--cyclocryopexia. Due to pain another surgery--tarzoraphia was indicated. In 2012 she underwent surgery at St. Elisabeth Cancer Institute in Bratislava--Nefrectomia transperitoneally l. dx., excision hepatis. Histological examination in addition to the primary papillary renal carcinoma--mucinous tubular T1 Nx Mx type, found the metastasis of malignant melanoma to the liver and right kidney. She underwent the diagnostic procedure to find the origo of the melanoma. The patient was subsequently admitted to our clinic with blind painfull eye for enucleation. During the surgery the was found retrobulbar tumor ingrowth. Histopatholigical findings confirmed malignant melanoma. Indicated was exenteration of the orbit due to malignant melanoma T4 N0 M2 stage in June 2012. After healing of the cavity she was recommended to design an individual prosthesis. After completing several courses of palliative chemotherapy during a recent review in January 2015 the patient is without recurrence of the melanoma in the orbit RESULTS: Histological examination confirmed malignant melanoma in stage G2, predominantly epithelioid type, spindle cell type in part B of pips, tumor fills the entire back and part of the anterior chamber, grows through the sclera and optic nerve is completely overgrown by tumor mass and spreads into orbit. The immunophenotype is suggesting a better prognosis (S100+, melanoma+, +HMB45, cyklin D1 3%, 10% of p53, Ki67 3%). Tissue eyelashes were infiltrated by numerous micrometastases. The patient after exenteration of the orbit after 3 months got an individual epithesis. Local orbit cavity is more than 24 months after exenteration without recurrence of melanoma. The patient is still undergoing outpatient chemotherapy and feels good. CONCLUSION: The treatment of malignant tumors of the orbit and the eye is difficult, in most cases surgical treatment is indicated, with the additional radiation therapy and chemotherapy. Malignant tumors at an advanced stage should to be solved radically. Exenteration of the orbit leads to produce a large defect in the orbit and this part of the face. Patients in the active age after surgery followed by facial defects after such procedures have disadvantage in work and thie defect leads to serious socio-economic challenges. Patients with individually made prosthesis comprising a refund of the eyeball and the surrounding soft tissues allow active life and full application of the private as well as professional life.