Omphaloliths are uncommon benign umbilical lesions caused by the accumulation of sebum and keratin into a stone-like concretion. Recognition of this entity can prevent unnecessary procedures and imaging studies for uncomplicated cases. We present three cases of omphaloliths from our department and review all 26 cases previously reported in the English literature with regard to modes of presentation, potential risk factors, complications, and treatment options to guide clinicians. The mean age at presentation was 48 years. Of the 29 cases, 17 (59%) were asymptomatic. Male patients presented at a younger age and were more likely to present with complications compared to females who presented at an older age with asymptomatic lesions (P=0.006). Features of patients described included dementia, hirsutism, a deep or narrow umbilicus, multiple nevi, obesity, and poor hygiene. Two patients developed overlying pyogenic granulomas. Removal of asymptomatic lesions was uncomplicated and done using forceps or following irrigation, with no recurrence. Complications, including localized abscesses and peritonitis, were associated in 41% of patients who were treated surgically; recurrence was noted in one patient. Removal of omphaloliths is recommended, once identified, to reduce risks of complications and patients should be encouraged to improve their personal hygiene.