Purpose: Total exenteration of the orbit with removal of the eye globe and surrounding tissues is most frequently indicated for malignant tumors. The indications for exenteration of the orbit for benign orbital lesion are rare. Not adequately treated infection of the orbit by systemic antibiotics can lead to destructive changes of soft tissues in the region of the orbit and partial exenteration with eyelid sparing technique is necessary. Design: Retrospective case series. Methods: Data of all patients between 2010 and 2018 who underwent exenteration of the orbit for periocular lesions infiltrating the region of the orbit were reviewed for patient demographics, previous treatment options, tumor localization and histopathologic type. Results: In group of 14 patients with periocular lesions total orbital exenteration underwent 12 patients (86%), in 1 patient biorbital exenteration was performed and in one patient orbital exenteration with eyelid sparing technique was performed. For 2 patients (14%) orbital exenteration was the first surgical procedure performed. In the group of total exenteration in 12 cases histopathologically basal cell carcinoma from eyelids was confirmed, in one case squamous cell epibulbar carcinoma was confirmed and in 1 case subtotal exenteration with eyelid sparing technique was performed—the authors reported the case. Case report of patient with long inflammation of the lacrimal pathway leading to orbital inflammation with eye globe destruction and partial exenteration with eyelids sparing technique was indicated. A 75-year-old man presented in 2014 with blepharoconjunctivitis and lacrimal sac inflammation of the left side. Treated in outpatient tract with local antibiotics, the drainage lacrimal system was transient. Few months later developed chronic blefaroconjunctivitis in cultivation result Citrobacter koseri positive was found. Patient was treated only with local therapy at outpatient tract again. In 2017 sent to hospital with painful eye-globe, visual acuity was no light perception. Computed tomography and magnetic resonance presented soft tissue mass extending along the medial orbit region in the m.rect. medialis and m. obliquus sup. and partly also m. rect. inf. space as a lesion of size 23 × 30 mm with a slight postcontrast homogeneous saturation and this lesion tightly fitted to the eyeball. Exenteration with lid sparing technique was performed. In 2019 after healing process patient got an individual epithesis. Conclusions: Basal cell carcinoma is the most frequent indication of orbital exenteration. Rarely is indicated subtotal exenteration with eyelid sparing technique for non-cancer reason as it was in our 1 case.