Ten percent of people are left handed, but a higher frequency has been associated with certain craniofacial malformations, such as cleft lip and unilateral coronal synostosis. The purpose of this study was to determine the frequency of left-handedness in patients with hemifacial microsomia (HFM). Patients with HFM were identified in our craniofacial database. Normal controls were recruited by local pediatricians. Data gathered included age, sex, and handedness (determined by writing and/or drawing); the orbit, mandible, ear, nerve, and soft tissue (OMENS)-plus score and side of involvement were tabulated for patients with HFM. Hand preference was compared between the groups using chi analysis; possible correlations were analyzed between handedness and age, sex, the OMENS score, extracraniofacial findings, and side of involvement. One hundred seventy-eight patients with HFM were identified; 92 (51%) were excluded. Of the 86 included, 48% were boys (n = 47) and the mean age at inquiry was 13.5 years. Predominant side of involvement was right in 49% (n = 42) and left in 38% (n = 33). Eleven patients (13%) had severe involvement of both sides. Expanded-spectrum HFM was documented in 41% of patients. Ninety-six children were in the control group; 44% were boys (n = 42), and the mean age was 10 years. The difference in age between the groups was significant (P < 0.05), but sex differences were not. Patients with HFM were more likely to be left handed for writing compared with the control group (26% vs. 11%; P < 0.05). The frequency was higher, 36%, in those with bilateral involvement (P > 0.05). There was no correlation with predominant side or OMENS score. This study confirms that this disorder affects cerebral lateralization.