Segmental odontomaxillary dysplasia is a rare disorder that unilaterally affects the maxilla causing facial asymmetry and presenting with hypertrichosis and hypoplastic or missing teeth in the affected area. Lip clefting has only been reported in one other case. We report a case of segmental odontomaxillary dysplasia presenting with hypertrichosis of the face, hyperlinear palms with faint erythema, and commissural lip clefting.