Myxoglobulosis is a specific type of mucocele consisting of mucoid material. It is characterized by opaque, transparent globules that resemble "fish eggs" or "frogspawns". It is generally diagnosed incidentally during an appendectomy or an autopsy. In this paper, we aim to present the case of a 58-year-old male patient who was referred to our hospital because of abdominal pain and loss of appetite. The patient underwent an appendectomy. Opaque intraluminal globules were found in the appendectomy material. The globules resembled pearls and they were 2-3 mm in diameter. After histopathological examinations, the patient was diagnosed with myxoglobulosis accompanied with acute appendicitis. According to our research, this is the first case of myxoglobulosis in our country.